Written by Paul Margulies, M.D, F.A.C.E., F.A.C.P. Medical Director, NADF.
Clinical Associate Professor of Medicine, New York University Medical School.
Q&A from March, 2013
Question: I read in an article that healthcare workers do not realize the urgency in treating an acute adrenal crisis, of fail to heed the requests of patients and family for hydrocortisone during a crisis. How do you recommend patients, and the caregivers of patients, communicate the urgency of an Addisonian crisis to healthcare professionals? Do you think it is better to use the phrase, “chronic adrenal insufficiency,” or, “Addison’s disease,” when talking to healthcare workers and requesting steroid administration?
Answer: This is one of the main goals of NADF. We try to provide education to patients, families and health care workers. I suggest that when a patient with Addison’s disease goes to an emergency facility with an acute adrenal crisis that they immediately tell the triage person that they have an Addison’s disease crisis, show them the NADF or similar card with instructions, and direct them to immediately give IV saline and IV hydrocortisone. If there is any delay, start making demands to see a supervisor or head of the ER.
Question: Can blast trauma cause adrenal insufficiency?
Answer: The answer is no. The adrenal glands are pretty well protected from outside pressure. Because they are glands, they are very soft, not stiff, so they would not be damaged by a percussive force. Bilateral adrenal hemorrhage would be necessary to cause Addison’s disease. When we do see this, it is a result of shock (low blood pressure) from surgery or sepsis from a severe infection, plus it usually is in the setting of an extra risk of hemorrhage such as use of an anticoagulant or auto-anticoagulants associated with lupus.
Question: I have secondary adrenal insufficiency, and for the last 10 years I have found that in a pinch, squirting the hydrocortisone solution of my crisis care injection into my mouth and swallowing it works if I can’t inject myself with the hypodermic needle. Is this a good way to treat an Addisonian crisis?
Answer: I do not recommend this approach. Since the purpose of an emergency injection is to provide a high dose when oral medication would be ineffective (especially if the person is vomiting), giving the injectable form into the mouth is not more effective than taking more pills by mouth. I suppose the injectable forms, like Solucortef would be absorbed, since it is a solution of hydrocortisone in sodium succinate. But if the individual is vomiting, the amount absorbed would still be uncertain. I would still suggest increased oral steroids for stressful events, IM hydrocortisone for emergencies when oral cannot work, and ER visits for IV saline and hydrocortisone when those treatments do not work. It is possible that squirting Solucortef into the mouth may help if there is no other steroid available, but it is not the ideal treatment.
Question: I have had Addison’s disease due to a bilateral adrenalectomy, and take 35 mg of hydrocortisone daily as a maintenance dose. I was put on a 12.5 mg dose of DHEA sulfate by an endocrinologist, but my family doctor found this level to be too low so I stopped taking it for a while. After checking with a specialist, my DHEA and cortical levels were (…) He told me to go back on my DHEA, but not to change my dose of hydrocortisone because taking the DHEA would bring my cortical levels to normal. Is this correct?
Answer: DHEA will not effect the cortisol level. It looks like the dose of hydrocortisone you are taking may be too high for a maintenance dose.
Q&A from June, 2013
Question: I’ve heard about something called “7 Keto DHEA”. Is it the same as regular DHEA?
Answer: 7 keto DHEA is a supplement sold on the market. It has no scientific research behind it. It is not a prohormone like DHEA and does not convert to androgens like DHEA. It is purported to have all sorts of metabolic benefits, none proven. I do not recommend it for anyone.
Question: I am a vegetarian, and an Addisonian. I’m worried about my potassium levels being too high or too low, and was wondering what: is the best way to manage them?
Answer: For a vegetarian Addisonian: Fruits are the best source of potassium, but there is a lot in many vegetables. As an Addisonian, you should be aware of how high your potassium might be on normal replacement therapy so you can avoid excess potassium intake.
Question: I don’t seem to be absorbing my oral dose of hydrocortisone well. At a high dose I seem to be ok, but when I taper back to a more normal dose, my levels are too low. My doctor is switching me to dexamethasone, but says there is nothing they can do if I have the same problem with it. What can I do?
Answer: I have a patient who has the same phenomenon. Basically, you need to take higher than average doses. The dosage can be adjusted to the point where signs, symptoms and blood studies are normal, even though the total dose may seem very high (compared to other people). Trying another oral steroid is OK too. I would suggest prednisone (plus fludrocortisone) or medrol rather than dexamethasone because of the shorter duration of action.
Question: I’ve been on prednisone non-stop for about 20 years for asthma. I was very slowly weaned to 7.5 mg every other day, a level I’ve been on for 3-5 years. Periodically I have to bump up the levels, and in the past 4 months I’ve had to bump them up and return to every day usage 3 times for asthma flares related to bronchial/lung infections. About 6 weeks ago I had a flare which required me to take the prednisone every day, in doses as high as 60 mg. It took about a month to get back to the 7.5 mg every other day. (I also have ulcerative colitis, but my primary medication for that is immuran.) Then I saw a new pulmonolgist. She asked me to reduce prednisone by 2.5 mg per week, becoming steroid free in a month. Her only warning about adrenal insufficiency regarded lightheadedness upon rising from a supine position. However, I wonder if that reduction is too fast, and if I should have blood tests to help guide the weaning. I am using 5 mg every other day now, but I have noticed weakness in my knees that I have associated with changes in steroid use in the past. I also have noticed new pain in my left hip, which I had not thought related, but now I wonder.
Answer: This is an example of the difficulty in tapering off long term steroids. I agree that the new pulmonary doctor may be too optimistic about your ability to taper and stop steroids after so many years of use and the frequent need to bump up to high doses. Going rapidly to every-other-day dosing in this situation is probably complicating the situation and contributing to the adrenal insufficiency symptoms (like the muscle weakness). Every other day dosing is useful in people who have been on steroids for a fairly short period of time, like a few weeks. In this current case, there is a great degree of adrenal suppression, so a slow taper of daily dosing has a greater chance of working. Once a dose of 5 mg is achieved, going down by 1/2 to 1 mg every 2 to 3 weeks may work better. Blood testing is not very useful, except for a morning ACTH once the dose is down to 2 or 3 mg, just to see if there is evidence of “awakening” of the pituitary. If ACTH is measurable, it is more probable that secondary adrenal insufficiency may resolve. Keep in mind that after 20 years of steroid use, many people have permanent secondary adrenal insufficiency and must settle on a baseline replacement dose of 4-5 mg of prednisone.
Q&A from September, 2013
Question: I’m an Addisonian, and also a sufferer of chronic pain. I feel like one can affect the other sometimes. Are the two in some way related, or can chronic pain lead to adrenal failure over a period of time?
Answer: Pain is a stress that can affect the management of Addison’s disease, and opiates prescribed for chronic pain can affect the metabolism of replacement steroids. But, pain does not cause the destruction of the adrenal glands.
Question: I was diagnosed as adrenal insufficient due to very low levels of cortisol in saliva and urine tests, and also have a hypothyroidism. I was on only a fourth of a pill of Nature’s Thyroid for 6 years. I could never raise it any higher without going into what I now know was adrenal crisis. Now that I am on Cortef, when I tried to take an eighth more, I went into crisis again. I asked my doctor to switch me to T3 only. He did it, but said that it isn’t good, because it will eat up all my cortisol and mess up my adrenals. What should I do?
Answer: First, I suggest a more accurate diagnosis of adrenal insufficiency than just salivary and urine cortisol levels. An ACTH stimulation test is the best way for most Addisonians. Second, I never use desiccated thyroid or pure T3 to treat hypothyroidism. The best therapy is levothyroxine (T4) with the dose appropriately adjusted based on symptoms as well as TSH levels. It is important to remember that the proper TSH level to aim for varies from person to person, so personal comfort and symptoms count. We don’t stop with a “normal TSH” because some will feel best with a TSH in the low normal range and others will feel best with a TSH in the mid or high normal range. Work with your endocrinologist!
Question: I have been using Prednisone for 35 years to treat my adrenal insufficiency. At a doctor’s suggestion,I switched to hydrocortisone, but it did not work well for me and I was tired all the time! I went along with a trial period of using hydrocortisone that the doctor suggested, though my fatigue symptoms continued. For 2 months I felt really lousy, and when I went back to Prednisone I felt perfectly fine within hours. However, doctors still sometimes insist that I should be on hydrocortisone instead. Is hydrocortisone really so preferable for treating Addison’s that I should be using it instead of Prednisone to treat my symptoms?
Answer: I suggest it is best to listen to your own body. Although hydrocortisone is more physiologically similar to normal glucocorticoid production than Prednisone, especially when given in divided doses with the highest dose in the early morning, if you feel better on Prednisone, stick with it. I suspect that your body has become used to the longer action of prednisone, so the dip in glucocorticoid activity in the afternoon and evening on hydrocortisone feels uncomfortable. As long as there are no Cushingoid side effects or sleep disturbances on the Prednisone, and the fludrocortisone dose is adjusted appropriately, there is no problem in staying on Prednisone.
Question: I have been seeing a doctor to try and diagnose a mystery illness I’ve been suffering for 2 years now. Some of the tests I’ve had done indicate adrenal insufficiency, but I’ve been told by an endocrinologist with my most recent tests that I am not adrenal insufficient. I’m very confused. Do some tests matter more than others for diagnosing adrenal insufficiency?
Lab results by themselves can be misleading.
Answer: The full clinical context; history, physical exam, medication history, and other basic lab results are all critical in making a diagnosis of adrenal insufficiency. Although I cannot give you a definitive answer, a normal response to an ACTH stimulation test is not diagnostic of adrenal insufficiency. If other aspects of your history points toward Addison’s disease, I suggest that your doctor add 21OH antibodies to test for autoimmune Addison’s. If your history is more suggestive of secondary adrenal insufficiency, this test is not useful.
Q&A from December, 2013
Question: I have had secondary pituitary adrenal insufficiency for the past 10 years. I have severe weakness in all extremities; the left worse than the right. My base dose of steroids is 6.5 mg of prednisone. I have had bilateral hip replacements and other orthopedic problems. My left fibula will dislocate from no trauma. My ankle strength is fair (3/5) and never seems to get any stronger. I was a physical therapist and was very active before my adrenal glands failed. I fall frequently. I cannot exercise heavily due to the Addison’s fatigue, severe asthma, and I am morbidly obese. Due to exacerbations from the asthma, I am on a roller coaster with the prednisone. Is it possible to increase strength through exercise taking a steroid for life?
Answer: The array of medical problems presents a challenge for you. The need for steroid replacement therapy itself does not prevent you from exercising and improving your strength. However, higher doses of steroids like prednisone can contribute to muscle weakness, so it is important to work with your doctor on finding the lowest dose that keeps you comfortable. The obesity is also a major issue. It exacerbates the arthritis and makes it harder to exercise. With less exercise, there is more weight gain. There is no magic formula, but try to reduce the dose of prednisone if possible, diet as well as you can, and try to find some form of exercise that will not cause further injury.
Question: I had a bilateral adrenalectomy (removal of both adrenal glands) over a decade ago. A CT scan of my abdomen revealed that one of my adrenal glands has grown back, after my cortisol levels started to rise. How is this possible?
Answer: Most likely, the surgeon left a small piece of one adrenal gland during the adrenalectomy. Over time, it could have slowly grown back into something functional. It sometimes happens.
Question: I was diagnosed with Addison’s in 1971. Recently, I underwent surgery to treat breast cancer, followed by radiation treatment. The cancer was luckily caught in stage one. I felt great until they put me on Arimidex. After that, I felt terrible and had every symptom of my Addison’s return even though I was on cortisone replacement. They took me off Arimidex, and put me on Tamoxifen. The same thing happened, and I was getting so sick that I went into adrenal crisis multiple times. Is this a known complication of those two drugs?
Answer: This is not a common event. Arimidex blocks aromatase, which is needed for the production of estrogen. Tamoxifen blocks the estrogen receptor. Neither should have a direct effect on cortisol metabolism. I suspect that the known side effects of these medications, which include aches and pains, might have felt like adrenal insufficiency. The pain itself will cause a need for additional doses of steroids.
Question: I had an ACTH stim test done to see if I was adrenal insufficient. I started at a cortisol level of 8, but dropped to 7.2. The RN said she had never seen a cortisol level drop before as a reaction to ACTH. I also have an aldosterone level of 3.8 (sitting) and a clean visual field test. I also had a recent severe drug allergy to aldactone. Is this normal for an Addison’s disease diagnosis?
Answer: If there was no recent exposure to steroids, the test shows an abnormal pattern, with failure of cortisol levels to increase after ACTH. That would be consistent with Addison’s disease. The fact that the level appeared to go down is not important. The two results are basically the same, and the failure to rise from a normal or low level is what is most important. But certain medications, including aldactone; which is used to treat hyperaldosteronism, can blunt an ACTH test.