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Questions and Answer Period from Cushing’s Patient Education Day, February, 2013

Question: Can Cushing’s be hereditary?

Dr. Findling: No, except in very rare circumstances. There are forms of adrenal Cushing’s that can be hereditary. In cases of bilateral adrenal hyperplasia, both glands are enlarged and have tumors and can be hereditary. This is very rare. Single adrenal tumors are not hereditary. There are some isolated reports of hereditary pituitary tumors, but they are very rare. There is one genetic mutation that can cause pituitary Cushing’s.

Dr. Swearingen: There is a condition called multiple endocrine neoplasia type I. It is a rare genetic problem and I have operated on such patients and they do have pituitary Cushing’s. But, they almost always have a family history of elevated calcium and renal stones, most probably because the parathyroid gland is affected.

Question: If one has had pituitary surgery, should we avoid taking steroids?

Dr. Woodmansee: A lot of that depends on where you are in your post op course. After surgery, you are taking steroids. After that, there are no absolute rules, but overall exposure is important.

Dr. Vance: Just to be clear, after successful surgery, most people have to take steroids for 6 mo to a year. But the general question, should I take steroids, depends on the reason. The worst thing I see is people who get steroid injections in their joints for joint problems. These people can look like they have Cushing’s since these drugs are very powerful and hang around for many months. We call that iatrogenic Cushing’s. If you have to have steroids to treat another medical condition, that’s one thing, but if it’s just to treat a pain in your hip, I’m not so sure. Again, there are some people who have to have steroids, like with severe asthama. A week or 2 of steroid treatment won’t hurt you, but the people we worry about are those that have to take steroids over a long period of time for severe asthma, rheumatoid arthritis, COPD and with some chemo therapy treatments.

Dr. Woodmansee: So, I don’t think you should be frightened if a physician is recommending steroids for some other type of condition. I think in general you need to be cautious and ask questions, because of exposure over time, rather than just saying sure. Of course there are those patients with pituitary insufficiency following surgery who have to take replacement steroids. Some 50% of Cushing’s patients are permanently on replacement following surgery.

Dr. Biller: If the question is “is it more dangerous for someone who had Cushing’s to take steroids”, then the answer is no. If they are in remission, they are just like anyone else. A short dose of steroids is fine. If the question is can steroids bring back a tumor that caused Cushing’s, the answer is no.

Question: Is there any indication that GH deficiency and pituitary tumors could be related?

Dr. Vance: No. There are many different types of pituitary disorders. Pituitary Cushing’s is a genetic mutation of one cell that keeps making copies of itself and forms a tumor. Deficiencies can be developmental and an event that happened during embryonic development in the womb. Sometimes there is a misstep in development of the pituitary resulting in a hormone deficiency. They are different pituitary disorders and we don’t think they are related.

Question: With an undetected pituitary tumor, why not just take out the adrenal glands?

Dr. Phitayakorn: Unfortunately, removing the adrenal glands means that the patient will be on life-long replacement medication. In addition, even though the pituitary tumor may be microscopic, after adrenalectomy, it will grow, sometimes more rapidly, because it is no longer suppressed by excess cortisol. Nelson’s syndrome could result.

Dr. Findling: Also, the quality of life on replacement cortisol is not the same as having normal adrenal glands, but it’s much better than having Cushing’s.

Question: Can you have pituitary radiation if a tumor is not visible?

Dr.. Vance: Yes, you target the whole pituitary gland, but about 70% of those patients do become hypo-pituitary and require replacement of other pituitary hormones.

Question: In a patient with pituitary Cushing’s, do you ever take out just one adrenal gland?

Dr. Vance: No. The adrenals are the cortisol factory. If you take out one adrenal the other will make excess. They used to do this years ago, but the Cushing’s returned in a few years.

Question: Does an adrenal tumor always make hormones?

Dr. Phitayakorn: A large portion of the population has a growth on the adrenal. Many of these are non-functional and do not need to be removed. It can become a concern when they grow, which could indicate cancer.

Question: Why do different patients need different doses of replacement medication?

Dr. Findling – Steroids are metabolized by the liver. Some medications can increase the clearance rate of steroids and some patients just normally have higher clearance rates. These patients can require much higher replacement levels. Also, some have different versions of the cortisol receptor that can make them more sensitive to steroids or more reresistant to steroids.

Question: If pituitary Cushing’s is know to be the cause of Cushing’s, why not just take out the entire gland?

Dr. Laws: We really don’t recommend that any more. If someone is really, really sick, then we usually do a bilateral adrenalectomy. Years ago when adrenal surgery was a very invasive operation, it was not an option for very sick patients. Now days with laparoscopic approach, it is an option. So, we don’t really remove the entire pituitary anymore. It would require life long replacement of lot’s of hormones and it doesn’t always cure the Cushing’s.

Dr. Swearingen – Removal of the pituitary requires total hormone replacement forever. If a good pituitary exploration doesn’t find anything in the pituitary, chances are that the tumor is not in the pituitary. Thus removal of the entire pituitary is not very often successful in curing Cushing’s.

Question: After weight loss from Cushing’s, is there a way to get rid of the excess skin?

Dr. Vance: The only way to accomplish this is surgically. Unfortunately, insurance generally will not pay for this because it is considered cosmetic. I have written many, many letters to insurance companies stressing that this is medically necessary and not the patient’s fault, but have not gotten the insurance companies to cover it.

Question: Do you set weight loss goals for your patients after successful surgery?

Dr. Vance: I don’t say, “loose 2lbs/wk. Some patients can do that and others can’t. Rather, I focus on cutting calories and increasing exercise. I can’t be rigid and set goals for some patients that aren’t attainable. Cut calories and exercise.

Dr. Laws: Your whole metabolism has been effected and you may not need as many calories as you used to. It takes time to recover a normal metabolism.

Dr. Woodmansee: It’s not realistic for me to tell my patients that I want you back to a normal weight in a year. I talk about diet and exercise and tell my patients that if you cut caloric intake by 500cal/day, that will result in about a 1 lb/week weight loss. It doesn’t come off automatically and that is the same as hypothyroid patients. Also, studies have shown that we underestimate what we eat. Having some type of structure is helpful.

Ellen: My weight has fluctuated. Don’t set yourself up for failure as it only brings on more stress and depression. I think it’s better to say I’m going to try to eat better, get some exercise. You’ve got a lot on your plate so you don’t need to create more stress for yourself.

Louise: Obestity rates in this country are astronomical. So many have eaten wrong all their lives even before Cushing’s and expect miracles after Cushing’s. We need to eat healthy with lots of fruit and vegetables. It takes work.

Dr. Woodmansee: In a pituitary patient, we also need to make sure that other pituitary hormones are normal as that can make a difference as well.

Ellen: Also keep in mind that some medications, including some anti-depressants, can cause weight gain. But, if you are depressed, use of these medications is worth it.

Question: What about exercise goals?

Ellen: Realize that you have lost a lot of strength. For me, at first a little hill was like climbing Mt Everest. It’s difficult to get back into shape. Water exercises are wonderful, but you don’t want to put on a swim suit. I say, just get over it. There are many others that didn’t have Cushing’s that look just like you. It’s not just about losing weight, but rebuilding your strength.

Louise: If you can’t do much, start someplace. For example, walk 5-10 min, then increase slowly. I don’t think a personal trainer is needed now. It’s a matter of just getting moving, do something.

Ellen: The best exercise is the one that you will actually do. Keep a journal; it can make you feel better about your progress. If you want to walk, for example to the library, when you finally get there, you can look back and see that a month ago, you only made it halfway. You can’t compare your progress with someone else’s.

Question: Does the level of cortisol make a difference in how long it takes to recover?

Dr. Laws: It’s not really the level of cortisol, rather it has a lot to do with how long you have had excess cortisol. If you’ve had it for years, the cortisol has done a lot of damage and it’s going to take more time to make progress.

Question: How many people get Cushing’s during pregnancy and if you are cured, does a pregnancy increase your chances of a recurrence?

Dr. Laws: I have seen numerous patients who developed Cushing’s during a pregnancy. Maybe it is some sort of trigger, but it’s not understood. I have not seen pregnancy trigger a recurrence.

Dr. Vance: I have only seen a recurrence after pregnancy in one ectopic patient.

Question: Do larger more invasive tumors recur more often?

Dr. Laws: No, larger tumors are more invasive and are more difficult to completely remove, but that doesn’t mean that if all of the tumor is removed that it is more likely to recur.

Question: How do you test for a recurrence?

Dr. Vance: I follow 24 hour urine cortisol values. ACTH is of no value as it is up and down all day.

Dr. Woodmansee: I agree. Night time salivary cortisol can increase due to exercise before the test.

Editor’s Note: Panel speakers were Dr. Beverly MK Biller (Mass. General Hospital, endocrinology), Dr. Whitney Woodmansee (Brigham & Women’s, endocrinology), Dr. Edward Laws (Brigham & Women’s, neurosurgery), Dr. Brooke Swearingen (Mass. General Hospital, neurosurgery), Dr. Mary Lee Vance (Univ. of Virginia, endocrinology), Dr. James Findling (Medical College of Wisconsin, endocrinology), Dr. Roy Phitayakorn (Mass. General Hospital, endocrine/adrenal surgeon), Ellen Whitton (CSRF Director), and Louise Pace (CSRF President).

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