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, 2010
Question: I have a son who has Addison’s disease. His current medications are Dexamethasone and Florinef. Can you tell me how significant contracting Herpes is to someone who has an Addison’s diagnoses? Thank you!
Answer: I assume we are referring to genital or oral herpes. It can be controlled quite well with antiviral medication, often given continuously, or sometimes as needed when there is a flare up. It should have no effect on the Addison’s disease because it rarely causes a systemic illness that would require a stress dose of steroids.
Question: A lady’s 15 year old daughter was diagnosed with primary Addisons on 9/12/08, and is on 22 1/2 mg of hydrocortisone and 0.1 mg of fludrocortisone acetate daily. The daughter has a behavior problem and also a learning problem. The mother wonders if there is a correlation with learning problems and Addisons Disease, as every time the daughter is given a higher dosage of hydrocortisone, her learning, speech, and behavior problems seem to go away. She is able to read, write, etc. Once the meds are lowered, she returns back to her prior state. This medication amount would be around 30 mg of hydrocortisone. The endo the daughter sees just stated that the high dosage has too great of side effects for her.
Answer: The brain responds to cortisol deficiency as well as excess. Changes is mood, behavior and cognition are often seen in these extremes. There is no basic learning disability due to Addison’s disease. The goal of replacement therapy is to restore function and health as well as possible, with the physical signs and symptoms as the best indicator. There is a risk that overdosing of replacement hydrocortisone may occur if short term learning and behavior is used as the central focus of treatment. Also, it is important to assess thyroid function. If abnormal, this will often cause cognitive impairment.
Question: I have Addison’s disease and Celiac disease. I also have high blood pressure. I take 20 mg of Bystolic for high blood pressure, but it is still high. My doctors are taking a wait and see attitude, but I am concerned. I thought that people with Addison’s disease had low blood pressure. I take 30 mg of hydrocortisone a day. I don’t take any Florinef. Do you think Florinef would help? Thank you.
Answer: People with Addison’s disease can have hypertension, usually essential hypertension as a familial tendency, or due to kidney disease. When this occurs, medications such as beta blockers or calcium channel blockers can be used, and the dose of mineralcorticoid is usually lowered or eliminated as long as the serum potassium is not elevated. Diuretics should not be used in Addisonians. If the potassium is elevated, low dose fludrocortisone can be added, with adjustments in the antihypertensive drugs to compensate. There is no reason to allow the blood pressure to stay high.
Question: I don’t know if you can help me find someone who has knowledge or experience with non-classical 21 hydroxylase deficiency and pregnancy. I am an adult endocrinologist and saw a woman with a history of elevated 17 hydroxyprogesterone levels that had been worked up and treated by her previous endocrinologist with decadron. She wants to become pregnant and wants to know if she should stay on or off the dexamethasone. Her first pregnancy ended with an atypical case of fetal demise at 22 weeks with atelosteogenesis. From what I can read, cases of overt classical CAH can deliver normal children without steroids, but some reviews warn that maternal androgens could virilize a female fetus. The woman is concerned that the dexamethasone might have caused the atelosteognesis. I have no experience with this clinical question. Do you have any people with experience I can call or correspond with?
Answer: Part A from Dr. Margulies: Although steroid use may be necessary to allow normal ovulation and conception, my own bias is to not treat non-classic CAH with steroids during pregnancy because the risk to the fetus is so small. Others may disagree with this. You could forward this to Dr. Speiser for her perspective. Part B from Dr. Speiser: The first question one must ask is what criteria were used to diagnose non-classic 21-hydroxylase deficiency. Many cases in which a patient is diagnosed elsewhere turn out not to have CAH at all. So, before answering the dex in pregnancy question, that must be clarified, and the ORIGINAL hormonal tests must be obtained. In animal studies, but not in humans, high dose exposure to glucocorticoids during early gestation may act as teratogens. I agree with Dr. Margulies that women with NCAH do not always need treatment to conceive, and once pregnant do not absolutely require prenatal glucocorticoid treatment, unless this same couple has a child with classic CAH. In the latter case, they should be offered genetic counseling and enter a clinical trial for prenatal diagnosis & treatment. Physicians who are inexperienced in this regard should not offer this treatment. See LWPES/ESPE Consensus Statement published in 2002 in J Clin Endocrinol Metab on which I am a co-author.
Q&A from June, 2010
Question: I’m a 55 year old female with Addison’s Disease. I had a lipoma removed from my back last month. I am exhibiting no symptoms of infection. My wbc was 15.6 last Tuesday. Yesterday it was 16.1. Other counts that changed from last week to this week are: RBC from 4.80 to 4.43; MCH 31.1 to 31.2; Neut. from 85.0 to 78.4; lymph 9.1 to 13.1; NEUT from 13.30 to 12.60; and MONO from .70 to 1.10. I can send you the normal ranges for this lab if you’d like. My physician wants to test me again in a month. If the wbc is still elevated, he will send me to a hematologist at the cancer center. The waiting is going to be very difficult. Would you have the same recommendation? Thank you.
Answer: Since you have Addison’s disease, one of the possible causes of the high WBC count could be a higher than normal dose of glucocorticoids since the surgery. If that is not the case, and it persists, further investigation is appropriate.
Question: My IGG levels are so low, that two doctors have said it is imperative I begin IV IGG treatments. It is not a easy decision to begin such therapy, and once begun, it is a process that involves risk and side effects.
Answer: Low levels of gamma globulin can contribute to frequent bacterial infections. This condition is not due to the Addison’s disease. Management with regular IgG infusions is indicated and should have no significant effect on the steroid management.
Question: How skewed (or not) are results of cholesterol blood tests due to prednisone? After getting diagnosed, the prednisone increased the combined cholesterol to over 200, something I’ve lived with for the past ten years. My internist prescribed Crestor, but I read that people with autoimmune disease shouldn’t take it. Which statin is better for adrenal patients?
Answer: High dose glucocorticoids can increase the blood level of cholesterol, but replacement doses for adrenal insufficiency will not cause this increase. Everyone should have regular check-ups that include testing for cholesterol and the fractions of good and bad cholesterol. If the levels are abnormal, and especially if there are other risk factors for heart disease, treatment with a statin is appropriate. The presence of autoimmune adrenal insufficiency is not a contraindication to using a statin. The specific choice is up to the treating doctor and the patient. Factors may include cost and any history of side effects.
Question: We have a member, an Addisonian since 1940, who has ìdiastolic heart failure.” The doctors are telling her she has to go on a low salt diet, and want to put her on Lasix. The last time she was given Lasix, she had lots of problems. Any advice?
Answer: Addisonians generally have trouble with diuretics because they are unable to handle the drop in blood volume produced by the diuretic. It is best to start with tapering or eliminating the mineralcorticoid (fludrocortisone), then, if necessary, switching from hydrocortisone to prednisone. In addition, there are other cardiac medications that can reduce the load on the heart without resorting to a diuretic. All avenues should be explored before adding Lasix.
Q&A from December, 2010
Question: This past year, for no known reason, my Addison’s has escalated with 9 adrenal crisis hospitaliztions. This March and April were back to back. We are looking for a root cause with abnormal results for both liver and lungs. I’m interested if NADF has any knowledge on this subject. Any information would be very much appreciated. Thanks so much!
Answer: I cannot speculate about the specific cause of the changes. Certainly any other medical problem, including liver or lung disease can have an impact on the metabolism of the replacement glucocorticoids, or produce extra stress that would necessitate extra steroid dosing. A thorough evaluation of any abnormalities is needed.
Question: I go to an endocrinologist about 3 times a year due to my thyroid. He does additional blood work. Since 2008 my ACTH has been below normal and continues to decline. The most recent test in May was <5. He says he doesn’t think it is a problem. He also suggested I could go to another doctor for further evaluation if I desired. I am not wanting to chase rabbits or spend a lot of time and money on doctors, however, I wonder if this is something I should look into to a greater extent.
Answer: It appears that your doctor includes an ACTH level as part of the routine blood testing. I think that is not appropriate. It is expensive and often misleading. ACTH levels are only useful as part of an evaluation of clinical features of adrenal insufficiency or cortisol excess. An afternoon plasma ACTH less than 5 is normal.
Question: I have been diagnosed with pheochromocytoma. Unfortunately, the I-123 MIBG that was performed at Mass General Hospital did not locate the tumor. My endocrinologist wants to wait for three months to re-test. My question is are there any other options? My BP has been all over the chart and now is relatively normal. The doctor thinks that the tumor is inactive at this point and that I should wait until the pressure is back up to the 200 level. This idea is very frightening to me because of the risks of stroke and heart attack involved. Are there any other courses of action rather than waiting for this to re-activate? Do you have any suggestions? This is a very frightening thing to deal with and I guess I am just searching for support or information. Thank you.
Answer: Most pheos are localized on MRI. If the MRI is negative and the MIBG scan does not localize it, there is no opportunity to operate. However, that does not mean that the condition should be ignored. If laboratory studies have confirmed an excess of catecholamines or their metabolites, medication should be used to control the blood pressure and pulse. The medications may include alpha blockers, beta blockers, and sometimes other blood pressure medications. As long as the blood pressure and pulse are OK, it doesn’t matter how long it takes to localize the tumor.
Question: While researching information for a new flyer an NADF volunteer was working on, she found information on Wikipedia that Addisonians may have raised calcium levels in blood. Have you heard about it?
Answer: Hypercalcemia can occur in Addison’s disease and I do see it frequently in my patients. It is generally mild and asymptomatic. I see it most often at times of stress when there is a relative insufficiency of hydrocortisone and may accompany mild hyperkalemia (high potassium). If it persists when adrenal replacement is in balance, it is a good idea to check parathyroid hormone just rule out hyperparathyroidism as a cause of the elevated calcium.
Question: My naturopathic doctor gave me a saliva adrenal test a few weeks ago. My cortisol levels were 1 for the morning, 1 for noon, less than 1 for the afternoon and less than 1 for midnight. When I called my endocrinologist and told them the results of the test, they told me that it was not reliable. Last month was the first time I have ever heard about Addison’s disease. I was diagnosed with hypoglycemia 24 years ago with my 1/2 hour fasting glucose level of 26. The doctor who diagnosed me left practicing medicine soon after that and no other doctor has done anything for it since. I read that in Addison’s disease low cortisol can cause hypoglycemia. I do have many symptoms that show I have problems with my adrenals. Is the saliva test that unreliable that my cortisol could be normal? Do you have any advice that could help me? Thank you.
Answer: Salivary cortisol tests are accurate if performed in a reputable clinical laboratory. There are many labs out there that are used by practitioners who are not physicians and I have seen many test results over the years that I do not trust. I would not recommend salivary cortisol testing to rule out Addison’s disease. Blood tests before and after ACTH (Cortrosyn) are the standard. The major use of salivary cortisol is in testing for Cushing’s syndrome, where midnight cortisol levels are abnormally high, and it is more convenient to do a saliva test rather than a blood test.
Question: My question is, could the adrenal gland have an adverse affect on the thyroid? I’ve been living with a thyroid condition for many years, but ever since 2008 my test results have been jumping every six weeks from one extreme to the other. In other words, either I’m under the range (0.03) or over the range (5.74). A pharmacist recently mentioned to me that they sometimes see T4 testing, which tells more of what’s going on with the adrenal gland. That made me wonder if I too could have some type of adrenal issue affecting my thyroxin levels. Icertainly would appreciate your thoughts. I’m beginning to feel my doctors in internal medicine are grasping at straws trying to figure this one out. Thank you again for anything you can offer.
Answer: There is no direct affect on the thyroid from any type of adrenal disease. However, it is important to remind anyone with adrenal disease that thyroid disease is very common. People with autoimmune Addison’s disease have about a 50% probability of developing autoimmune thyroid diseases such as Hashimoto’s thyroiditis or Graves’ disease. When thyroid disease and adrenal insufficiency co-exist, the management of both must be coordinated. When severe hypothyroidism and adrenal insufficiency are diagnosed together, it is important to treat the adrenal insufficiency first, otherwise the thyroid hormone can worsen the adrenal symptoms.
Question: In January 2010 I had surgery for Lichen Sclerosus (autoimmune). I was increased on my high potency topical steroids for about 3 months prior to surgery, then told I needed to stop after surgery to heal. I crashed. 3 different specialist have said in conversation (not diagnosis) that I have Addisons disease, after telling what happened. I have felt this is true since 2006 after a different surgery. Since I cannot seem to get referred to an endocrinologist, I have used Dessicated Adrenal for 2 years now. I had also tried stopping the dessicated adrenal when they told me no steroids after the surgery. That also contributed to my crashing (severe muscle weakness, salt cravings, extreme fatigue, vomiting, confusion). My question is, does anyone know if this supplement can give false normal cortisol levels, as it has some steroidal effects. I do know I cannot function without it. I am afraid when I finally have my endo appointment in Feb. that my levels may be false normals, and I will not get proper treatment. I also take Rehmannia periodically, as it has an immune suppressing activity to it. Thank you.
Answer: Over the counter dessicated adrenal extract is unregulated. The FDA has inadequate resources to monitor ingredients. It is not legally allowed to have any significant hormonal activity, but who knows? I do not recommend it for anyone at anytime.
Question: I am very concerned about my forty year old granddaughter – she appears to be following in my footsteps healthwise. At age 39, after gaining from 135# to 190#, I was diagnosed with Cushing’s Syndrome. I had a bilateral adrenalectomy. I did quite well for three years, then the symptoms reappeared and I gained all the weight back, plus the widow’s hump. I was sent to an endocrinologist & after three months they said I had more adrenal glands growing in my body. I was so weak I couldn’t even shake hands. The doctor suppressed the glands where ever they were, and I’m now regulated on 20 mg am and 5 mg pm. I’m still weak and walk with a walker. My granddaughter has all the symptoms – even fell down a flight of stairs while holding on to the rail, as I did many years ago. She told her GP who ran a 24 hr. urine & and I don’t know what other lab work. My granddaughter has gained up to 200 lbs – mostly face and trunk of the body. Legs & arms are thin. She also broke her shoulder of the arm when she was trying to break her fall. I do not want to interfere but I’m very concerned. I hope and pray Dr. Margulies can study this and I’ll provide more info if he needs it. My materal grandmother weighed 300 lbs, Please forward this to Dr. M. for me. I will be forever grateful.
Answer: There are some familial forms of Cushing’s syndrome, especially when they are caused by bilateral adrenal nodules. Your granddaughter should definitely have a thorough evaluation.