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, 2015
Question: I was diagnosed with Addison’s in 1994. It’s been noted in recent years that my bloodwork is showing slowly decreasing potassium results. It used to read at 4.0, but last time was at 3.5, a little below the bottom of normal range. That last time, my sodium was also low end of normal. is there something I should be considering to explain this?
Answer: The most likely cause is a very slight imbalance in mineralocorticoid intake vs fluid intake. Aside from just looking at the electrolytes, look at the total picture. Look for evidence of a slight excess of mineralocorticoid, such as a slight increase in blood pressure, slight ankle swelling and check the plasma renin (if it is low, it suggests too much mineralocorticoid). Remember that mineralocorticoid effect comes primarily from fludrocortisone, but also from hydrocortisone. Also, look for hypothyroidism, since this can contribute to increased water and dilution of sodium and potassium. Finally, look at any other medications that might have changed.
Question: I am a diabetic with Hashimotos disease. I recently had my thyroid gland taken out. My doctors say I will very likely lose my adrenal glands as well. Is this also your opinion?
Answer: That’s a bit pessimistic. Autoimmune adrenal insufficiency is quite rare compared to the incidence of Hashimoto’s thyroiditis or diabetes, so it is still very unlikely to occur. If the diabetes is type 1, I would say the possibility is greater (if type 2, there is no genetic influence), but still very small. The only other factor that might push up odds of getting Addison’s disease is a family history of Addison’s. In any case, if the doctor really is worried, he can check the 21OH anti-adrenal antibody level. If it is positive that would be significant.
Question: I had an 8cm pheochromocytoma removed in 2 years ago, and a second one grew but only became functional 16 months back. It showed a 4 times higher than normal cathecholomine output, and BP out of control. The pheo is still really tiny; it has only grown to 1.1 cm last it was checked. The doctors say we should wait for it to grow before removing it, because living on steroids reduces quality of life. The symptoms from pheos however have been very bad. Are there any better treatments you can suggest?
Answer: With the occurrence of a second pheochromocytoma producing significant symptoms and blood pressure elevation, surgery remains the best approach. The fact that the second tumor is small may allow the surgeon to attempt a laparoscopic resection of the tumor and try to leave the rest of the adrenal gland intact, preserving adrenal cortical function. Even if that is not possible, surgical Addison’s disease may be preferable to poorly controlled catecholamine excess.
Question: Do you find those of us who take prednisone gain more weight than those taking hydrocortisone?
Answer: No. It’s a matter of dosage in glucocorticoid equivalents. 5 mg of prednisone = 20mg of hydrocortisone.
Question: When I was diagnosed with Addison’s disease, I was told not to take aspirin because salicylates affect cortisone’s performance. I recently became aware of other things that contain high salicylates, like certain fruits, vegetables, nuts, and herbs. Also, there are other over the counter medicines that contain salicylates. Should I be concerned and take special precautions to eliminate or reduce the use of these sources of salicylates?
Answer: There is nothing wrong with your use of any of the foods listed or any over the counter products that include salicylates. They will not affect your adrenal therapy.
Q&A from June, 2015
Question: I am a nurse, with a patient who has an allergy to hydrocortisone, but can tolerate prednisone. Because of their allergy, I am worried about giving them a prescription for hydrocortisone for an emergency injection. Are there alternative preparations for an emergency injection?
Answer: There are two non-hydrocortisone preparations available for injection: methylprednisolone (Solu-Medrol) and dexamethasone. These glucocorticoids have very little mineralocorticoid activity (especially dexamethasone), so it is not the same as giving IM hydrocortisone in an emergency. I would suggest a thorough evaluation of the “allergy” to hydrocortisone, since it is quite rare.
Question: Colonoscopies can be a terror to many. Dehydration and nausea are big factors, and prevents many from going through with them. I’m very concerned about the effects of both the preparation and procedure causing someone with adrenal insufficiency to go into crisis. What sort of precautions can patients take to help them?
Answer: The cleanout protocols have changed and are now gentler. There is no need for inpatient colonoscopy unless there are other significant medical problems, like severe heart disease or a history of complications from previous colonoscopies. As always, I recommend individualization of steroid management, so if the prep causes nausea or severe cramps, extra hydrocortisone should be used to cover those symptoms on the prep day. However, if the cleanout just causes the usual diarrhea, and appropriate fluids are used to avoid dehydration, a normal steroid dose that day will be sufficient. My recommendation for a slight extra dose on the morning of the procedure stands, although even that is probably not needed in most cases. But again, a higher dose can be used if there are other stressors. I continue to recommend that the anesthesiologist be prepared to give IV hydrocortisone if needed.
Question: My sister, who is 76 years old and has been living with Addison’s since she was 13, has recently been showing signs of dementia. According to Medline on the internet, metabolic dementia can be caused by Addison’s. Is this true and, if so, what can we do about it?
Answer: Although Addison’s disease can contribute to dementia if undiagnosed or untreated, it would be an unlikely cause. If she has been well treated all these years, I would consider Alzheimer’s disease as a likely primary cause. I suggest a neurologic consult to rule out other disorders, thyroid function tests to rule out hypothyroidism, and supervision of medication compliance to ensure proper steroid replacement.
Question: Doctors are saying that I can have chronic steroid myopathy with adequate replacement of steroids. They say steroids are synthetic, therefore will have side effects no matter how low my dose is. Other Addisonians have said that hydrocortisone mimics normal cortisol production, therefore we should not suffer from side effects. What should I believe?
Answer: I disagree. The synthetic aspect is not a factor, although long duration of activity could be, especially with prednisone. Steroid myopathy is generally seen in people on chronic doses of glucocorticoids that exceed the normal physiologic replacement dose. That is why it is a feature of Cushing’s syndrome. For most people, doses of prednisone over 5 mg daily or hydrocortisone above 30 mg may put a person at risk for this, as well as the other complications of Cushing’s. For individuals with adrenal insufficiency who take replacement doses, steroid myopathy should resolve over time unless other diseases or medications complicate the recovery. If myopathy does persist in the absence of other factors, it may suggest that the maintenance doses are actually still too high, and it might be worthwhile trying a careful reduction in dosage. If prednisone is used, it might be switched to hydrocortisone, with its shorter duration of activity.
Q&A from September, 2015
Question: I am a 27 year old Addisonian male, and was diagnosed at the age of 13. Since then, I have been really skinny. I am currently 6 feet tall, and weigh 135 lbs. I have tried almost everything to put on muscle, but have not been able to. I read that when diagnosed at a young age, there should be testosterone and growth hormone replacement therapy. But I never had this. Both my father and brother are much heavier with muscle than I am. I was wondering if this could be caused by low testosterone, and if so, would my Addison’s have anything to do with that?
Answer: You should get a thorough endocrine evaluation, as you raise some appropriate issues. If you have typical autoimmune Addison’s disease, it usually would not include growth hormone deficiency, and you are 6 feet tall. Testosterone deficiency is another matter, and should be investigated. Of course, your dosage of glucocorticoids could be the basic issue. Your endocrinologist should evaluate the adequacy of your dosage and consider a trial of an increase.
Question: I have CAH and take fludrocortisone, but it doesn’t help me entirely with losing salt. I am losing way too much salt, and taking more fludrocortisone doesn’t help me feel better. This happens every few weeks, and I feel like I might go into an adrenal crisis, but then I’m fine for a while. According to the doctors, this isn’t due to my thyroid. What should I do?
Answer: You clearly need a more experienced endocrinologist to work with you. There may be something else going on besides the salt losing CAH. It takes a clinician to sort it out. One specific suggestion is to check your plasma renin as a guide to hydrocortisone dosing.
Question: Having been an Addisonian for 42 years, I have a question about fatigue. When I was working 40 hours a week, cooking and cleaning for a family of seven, I was taking 5 mg. of Prednisone a day and doing fine. After retiring I had increased the dose to relieve arthritis to the point my muscles became weak. Now that I am 80 years old and not real active, I am still taking the 5 mg. daily and experience muscle weakness and fatigue. Could these symptoms be caused by more Prednisone than I need at this point? If possible, how should I proceed?
Answer: Yes, the use of higher doses of prednisone over many years to treat the symptoms of arthritis could have caused atrophy of the muscles, especially the proximal muscles such as the thighs, leading to difficulty getting up from a chair or climbing stairs. At this point, even 5 mg may be too much if your weight has diminished. It is possible that a slight taper may help with muscle strength, but it will be a slow process. Before reducing the dose, a full evaluation with your doctor is necessary, to make sure there are no other medical problems that might make a prednisone reduction inappropriate.
Question: I am aware of recommended surgery protocol for adrenal insufficient patients. Are there recommendations for the 2nd and 3rd day post-surgical, bilateral knee replacement therapy as one begins ambulation?
Answer: The speed of steroid taper post-op depends on the severity of the post-op stress, which usually means pain. After knee replacement, narcotics are typically used, and these affect the steroid dosage indirectly. It would be common to need a double oral dosage for the first 2 or 3 days post-op. If pain is well controlled, a taper to 1 and 1/2 of the usual oral dose may be needed for several more days until pain is limited. If there are any surgical complications, such as infection, a return to higher stress doses would be needed. When in doubt, if any adrenal insufficiency symptoms occur, such as nausea or dizziness, continue to take extra glucocorticoids.
Q&A from December, 2015
Question: I am 67, have had well controlled Addison’s and Hashimoto’s for about twenty years. I have been taking 37.5 mg of cortisone acetate daily until it became unavailable last spring. At that time I began taking hydrocortisone and experiencing exhaustion, severe muscle pains in my legs, burning pain in my feet and jaw pain.
As of last December I have once again been taking cortisone acetate. The severe leg pains and jaw pains have subsided, for the most part… replaced by a feeling of heaviness in my legs when I am up and about for more than an hour. Additionally, at my annual visit with the endocrinologist, it was discovered that I no longer have ankle reflexes (my reflexes have always been brisk) and decreased sensation in both feet. My B12 level is 300 (range 200-1200). An EMG showed slight slowing in two nerves bilaterally, but nothing else.
Do individuals with Addison’s need more B12? Have other individuals experienced these side effects with HC?
Answer: Addison’s disease does not directly cause vitamin B12 deficiency, but there is an increased incidence of pernicious anemia caused by anti-parietal cell antibodies that then diminish stomach acid, resulting in low B12. If the serum B12 level is normal, this process is unlikely, but I would include a blood test for the anti-parietal antibodies. The symptoms from the shift from cortisone acetate to hydrocortisone and back suggest that the dose of hydrocortisone was inadequate. The equivalent dose is 30 mg, but some people do need more, so if a shift back to hydrocortisone becomes necessary, I suggest going up to 35 mg. Also, keep in mind that low B 12 and diabetes are not the only causes of peripheral neuropathy. The neurologist might need to do other testing to sort this out.
Question: I heard on a radio station about a doctor who said not to drink caffeine for at least an hour after waking up because it lowers cortisol levels. Could caffeine then affect adrenal insufficient patients’ replacement cortisol levels?
Answer: I’ve never heard of any clinically significant effect of caffeine on cortisol levels.
Question: I have pituitary secondary adrenal insufficiency. My 8 am cortisol level is always 0.8 (range 4-22). My renin level is almost always normal, but this month it was 0.5 (normal upright sitting/non-hypertensive is 0.65-5.0 ng/ml/h/). My endo said my adrenal gland must be doing something with these numbers on the renin activity. Presently I am on 8 mg of prednisone and trying to wean down to my base dose of 6 mg. I am also on 0.2 mg of florinef and my potassium level was 3.4 mmol/L, and I take 20 mcg a day and now my endo doubled it after the blood test. First, do you agree? Second, what is the relationship between cortisol and renin activity levels?
Answer: The lab results mentioned, which were taken while on prednisone and Florinef®, do not give any useful information about any residual glucocorticoid or mineralocorticoid function. The AM serum cortisol would be expected to be very low in secondary adrenal insufficiency. Most people with secondary adrenal insufficiency retain mineralocorticoid (aldosterone) production, and do not usually need fludrocortisone (Florinef). However, some people do need it. The best clue is elevated serum potassium and an elevated plasma renin. Since you are on Florinef, I assume it was added for that reason. Once on Florinef, the dose is adjusted to keep the blood pressure normal, as well as a normal potassium and renin. Since the dose of Florinef 0.2 mg is bringing the potassium and renin down to a low level, the treatment is to reduce the dose of Florinef, not increase the potassium intake.
Question: I had a bilateral adrenalectomy to treat Cushing’s disease after having a pituitary tumor removed didn’t work. However, having a history of kidney stones I have had several CT Scans in the past four years. To my surprise each report has indicated that my left adrenal gland is unremarkable in appearance. The most recent CT scan done on January 9th also indicated my left adrenal gland is there. My internist questioned the radiologist about this, explaining that I had both adrenal glands removed. The radiologists insisted that I have a test to determine if residual adrenal gland tissue has grown back and to see if it is functioning as an organ. Can this be? I have already done a particular test where my endocrinologist had me take dexamethasone and do a 24 hour urine test to see if cortisol was present, but my internist said that is not an adequate test to determine if the organ or residual tissue has, in fact, grown back. Can you recommend the correct test to find this out?
Answer: The endocrinologist is right. If there is functioning adrenal cortical tissue, the 24 hour urine cortisol while on low dose dexamethasone will show measurable cortisol. Since the pituitary surgery did not cure the Cushing’s disease, there is still some ACTH production which would stimulate any residual adrenal tissue to make cortisol. Frankly, I wonder if this exercise is really necessary. If you are doing well on replacement steroids and there is no clinical sign of cortisol excess, the imaging appearance is not very important unless it shows a definite tumor.