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, 2006
Question: Is it common for Addisonian’s to experience body aches, cramping and muscle spasms? What might be the cause? Is there any feedback data concerning successful treatment modalities from patients?
Answer: Untreated or poorly replaced Addison’s disease can cause muscle spasms and cramps, especially in the abdomen. These symptoms usually resolve promptly with hydrocortisone because they are primarily due to the electrolyte abnormalities of untreated adrenal insufficiency (high potassium and low sodium). If a treated addisonian continued to have muscle cramps or aches when all the other symptoms have resolved, other causes should be sought, especially hypothyroidism which is very commonly associated with Addison’s disease.
Question: I have had Addison’s disease for 20 years. I am needing any information anyone can give me on joint pain, osteoporosis and long-term steroid use. Since being on steroids for 20 years, I have severe back (discs) and joint pain to the point I can’t stand or sit for even short periods of time. I am needing proof that the steroids could have caused this problem. Can you please help – do you have any info on this or suggested books I could read or recent studies on long-term steroid use.
Answer: There is a lot of confusion and misinformation about steroids and arthritis as well as osteoporosis. High dose steroids, as used to treat illnesses like asthma, rheumatoid arthritis and ulcerative colitis, will cause osteoporosis (bone thinning) and increase the risk of fractures. This can now be prevented with drugs like Fosamax and Actonel. Replacement steroids to treat Addison’s disease are much less likely to cause osteoporosis because a lower dose is used that is meant to replace what is missing from the adrenals. The only study of osteoporosis in Addisonians showed only a mild tendency in men, not women. Arthritis is not caused by steroids. As noted, it is used to treat the inflammation of rheumatoid arthritis. The only joint complication of steroids is the rare occurrence of avascular necrosis of a joint, especially the hip, where the circulation to the bone is damaged, resulting in a sudden loss of a part of the bone within the joint. Avascular necrosis is most commonly seen in people given high dose steroids to treat medical conditions, but has rarely been reported in Addison’s disease. Most likely the back pain and other joint pain you have is due to other factors. It is unlikely to be due to the Addison’s treatment.
Question: My father has been on some form of steroid everyday for the past 20+ years for rheumatoid arthritis. Is it possible to get completely off this drug after so many years?
Answer: Tapering off long term steroids used to treat chronic illness like rheumatoid arthritis is usually difficult, and is harder with very long term use like this. The first issue is what is the dose of steroid used. If prednisone over 5 mg or Medrol over 4 mg is used for many years, the adrenals have been totally suppressed all that time and are less likely to work again. Another issue is the state of the disease being treated. There is no point in reducing the dose of steroid if the disease is still very active, unless another drug can be substituted. Once the taper is started, if the disease symptoms flare, then the steroid dose must be increased again. Even if tapering is successful, it will take about a year for the adrenal responsiveness to be normal and during that year, steroids will be needed to handle stress. Most of the time, after 20 years of steroid use, a stable replacement regimen like prednisone 5 mg is the easiest solution.
Question: My daughter was recently diagnosed with Schmidt’s syndrome. In addition, she has had a headache for over a year. The headache gets somewhat better at times, and is worse at others, but never goes away. We still do not have a diagnosis on the headache, although tension seems to be a significant contributor. Due to the headache pain and the fatigue associated with Addison’s, she has become depressed. Could the Addison’s and Hashimoto’s be causing her headache? She had scoliosis surgery a year ago in November and now has two Titanium alloy rods in her back. Could a metal allergy have caused the onset of Addison’s? She developed low blood pressure and orthostatic intolerance two weeks after surgery. Could this have actually been an Addison’s crisis and we are just fortunate that she still was making enough Cortisol to get through the surgery? Could a difficult menstrual cycle be stressful enough to require an extra 5mg of cortisol?
Answer: Depression is common in individuals with inadequate treatment of both adrenal insufficiency and hypothyroidism, but it is also common in the general population and in the setting of chronic illness. Headache can also be seen in hypothyroidism. Metal allergy is not a known cause of adrenal insufficiency. Certainly, hypotension after surgery may have been a sign of adrenal insufficiency at that time. Yes, menstrual cramps can be severe enough to require extra steroid treatment. Try a dose of ibuprofen at the onset of the cramps to minimize the pain.
Q&A from June, 2006
Question: I have Addison’s disease and would like to know if I could/should have tetanus shots. A few years back when I was working in the public school system and could get different shots through the schools I was told that they would not give me the tetanus shot because of the Addison’s disease or the cortisone. I don’t remember which. Is there any truth to this? Have a Wonderful Day!
Answer: There is no reason an Addisonian should not have a tetanus shot.
Question: Would you please address in an upcoming newsletter the effect of drinking and having Addison’s disease. Thank you.
Answer: Moderate alcohol consumption is not a problem in Addison’s disease. The metabolism of alcohol is not affected, and all the side effects of alcohol on weight, behavior, cognition, etc are the same as everyone else. Excess alcohol intake that leads to cirrhosis would cause the same issues for an Addisonian as others, but the fluid and electrolyte complications would be more serious and life threatening.
Question: What educational disablities do Addisons disease have?
Answer: The answer is none. Obviously, undiagnosed Addison’s, or poorly treated Addison’s will cause significant symptoms, including cognitive difficulties. However, once recognized and treated, there are no barriers to learning in a person with stable adrenal insufficency. Although education was not specifically queried, re-read the “Social Aspects” section in the NADF North American Survey. (Survey can be found at the NADF website: http://www.nadf.us/)
Question: Hello. I have a question I can NOT get a straight answer to! Can you please help? My registered dietician friend says that it will be almost impossible for me to lose weight (as measurable on the scale) due to the fact that the Florinef I take (0.5 of a tablet in AM) causes me to retain water. She said I have to have my body composition checked to see if my body fat reduces by dieting. But in any case, even if my body fat reduces, I will still weigh the same due to water retention. Is that true?
Answer: Your nutritionist is mistaken. Florinef (fludrocortisone) does cause fluid retention by helping the kidneys hold onto sodium and excrete potassium. The purpose of the medication is to help the Addisonian maintain a normal blood volume and avoid low blood pressure and any fall in blood pressure on standing that would result from the deficiency in the hormone aldosterone caused by the Addison’s disease. Florinef replaces aldosterone. In contrast to hydrocortisone, the Florinef does not cause any fat production. If you are on an ideal dose of hydrocortisone as well as Florinef, you can and will lose weight if you diet and exercise. If you take an excess of Florinef, you may have excess fluid retention, resulting in ankle swelling and high blood pressure.
Q&A from September, 2006
Question: I would be grateful if you could you tell me what type of an impact continued severe emotional stress can have on someone suffering from secondary adrenal deficiency caused through the presence of a pituitary tumor.
Answer: Emotional stress can cause significant symptoms of adrenal insufficiency in primary as well as secondary causes. Under normal pituitary-adrenal conditions, emotional stress would tend to promote an increase in ACTH and cortisol production. Since this does not happen in adrenal insufficiency disorders, the body feels like there is a lack of cortisol. Typical symptoms would be fatigue, lethargy, loss of appetite, weakness, dizziness and depression. Just as in cases of physical stress, one needs to remember to increase the dosage of glucocorticoid replacement. Laboratory tests are not very useful in this circumstance. If prolonged use of higher doses leads to signs of cortisol excess, then cut back to the lowest dose that keeps you feeling better. Also, try to deal with the cause of the emotional stress, if possible.
Question: I just read a newspaper article that says the Advisory Committee on Immunization Practices, a government advisory panel, recently recommended that Americans 60 and older get vaccinated against shingles. Zostavax is a souped-up version of Merck’s chickenpox vaccine for children, with a live virus that is 14 times more potent. My question: Is this live virus safe for those with Addison’s Disease?
Answer: I do not think there is a safety issue with Addison’s disease. It is important to remember that Addisonians are not more prone to illness or side effects from medications than anyone else – they simply are more likely to feel sicker and may have adrenal insufficiency symptoms requiring extra steroids if they should get an illness, or suffer a side effect, such as fever from a vaccine. If an Addisonian should happen to get shingles, the effects of the illness can be quite serious, so the risk of a slight fever from a shingles vaccine may well be worth it.
Question: Does Addison’s disease impact on sexual drive and sexuality?
Answer: Most people with Addison’s disease have perfectly normal libido (sex drive) and sexual function. Men usually have normal erections and ejaculation, and women usually have normal orgasms. When sex drive is lacking, it may be due to sensitivity to the loss of dehydroepiandrosterone (DHEA), measured as DHEAS in blood tests. This is an adrenal androgen, and it is missing because the entire adrenal cortex is lost in Addison’s disease. I normally recommend that people with Addison’s disease try replacing this hormone with over the counter DHEA pills – 25 mg per day for women, 50 mg for men. Sometimes, but not always, this can help. It also may provide a slight sense of improved well being in Addisonians generally. If this does not help, men should have their serum testosterone checked. Also, keep in mind that sexual function can be affected by any other medical problem that might coexist with the Addison’s disease, especially thyroid disease and anemia. Other medication can have an effect, such as beta blockers. Finally, don’t forget psychological factors. All together, though, there is no reason an Addisonian shouldn’t be able to have normal sexual function.
Question: A member with auto-immune Addison’s disease was recently told by a psychiatrist that her depression is caused by her hydrocortisone. (She is on 30 mg. Cortef a day, no Florinef.) She would like your opinion.
Answer: There is a complex relationship between cortisol and depression. Excess cortisol seen in Cushing’s syndrome can cause mood changes including depression. Sharp and sudden excess cortisol from high dose steroids may cause dramatic mood changes including manic behavior and even psychosis. Low levels of cortisol in undiagnosed or inadequately treated Addison’s disease will often contribute to a sense of depression along with the other symptoms of adrenal insufficiency. Restoring the glucocorticoid level to normal with hydrocortisone will usually improve mood substantially. Basically, aiming for ìnormal” with hydrocortisone treatment should include mood in addition to the typical physical features that are monitored. However, clearly some people with Addison’s disease may have a coincidental clinical depression that cannot be managed by simply manipulating the hydrocortisone dose. Many Addisonians in excellent control with hydrocortisone and fludrocortisone also benefit from psychotherapy and antidepressant medication.
Q&A from December, 2006
Question: Are flu shots made from dead or live virus?
Answer: Current flu shots are purified surface antigen made from killed virus. Everyone should get one, and there is not increased risk from the immunization to people with adrenal insufficiency.
Question: Why do some secondary adrenal insufficient patients (either from pituitary non-function or adrenal atrophy from long-term cortisol prescription use) end up needing to take aldosterone replacement medication?
Answer: Aldosterone is primarily regulated by the kidney. When blood volume drops, the kidney makes renin, which then stimulates the production of angiotensin, which is metabolized in the lung, and then stimulates the adrenal to produce aldosterone and increase sodium retention and potassium excretion and increase blood volume. This mechanism usually does not require the pituitary, and therefore most people with secondary adrenal insufficiency (who lack ACTH) have only cortisol deficiency, but still maintain adequate aldosterone production, since their adrenals are intact. However, there are some people (about 10% of the population) who do seem to need ACTH stimulation to maintain their renin-aldosterone balance. These people wind up with high potassium levels despite prednisone treatment, and they do respond to fludrocortisone (FlorinefÆ), or may be managed with hydrocortisone in place of just prednisone alone.
Question: I was wondering it there is a healthy weight maintenance diet that people with Addison’s should follow, especially when hypoglycemia is a concern. If you could send me any information that would be wonderful.
Answer: The best diet for people with Addison’s disease is a well balanced nutritious diet with three meals per day, adequate complex carbohydrate and protein, and adequate salt. If hypoglycemia is frequent, there is probably a need for a higher dose of hydrocortisone, but adding small snacks between meals can help. Addisonians must drink enough water to avoid dehydration and low blood pressure, and increase fluids with exercise or in hot weather. Maintaining a normal weight is important for everyone, including people with adrenal insufficiency. Beyond these generalities, there is no specific ìAddison’s” diet regimen.
Question: I had Addison’s for 11 years, and now have hypothyroid condition. Adding Synthroid, I am getting CRAZY. Whenever I have to raise steroids (infection, stress, etc.) it causes me to go very HyPOthyroid. Then when I lower the steroids & taper down, I go very hyPERthyroid!!”
Answer: As you know, hypothyroidism and Addison’s disease will commonly occur together, and there is usually no conflict. Each replacement must be handled separately. Typically, hypothyroidism will start with partial insufficiency and a mild replacement dose, but will usually slowly progress with further loss of thyroid function, and the replacement dose will increase to fill the gap. In contrast, when Addison’s disease is diagnosed, a full replacement dose of glucocorticoid and mineralocorticoid is necessary right away, and the adjustment in dose is based on body size, activity, and stress levels in daily living. Doses of glucocorticoid, such as hydrocortisone, have only a minor affect on thyroid hormone levels. Often, high doses make one feel ìhyper” without necessarily changing blood levels, and may be followed by a feeling of let-down that feels like ìhypo”. On the other hand, since higher doses of hydrocortisone are given for stressful illnesses that may cause fatigue that feels like hypothyroidism, and are then relieved at the time the steroids are tapered, which may contribute to the inverse symptoms you describe. The best way to check is to look at the blood levels, especially of TSH as a guide to thyroid replacement.