I was looking for information on weaning off of steroids and came across this article and survey done by the CSRF. I especially enjoyed reading other Cushing's patients experience with tapering. I am still on 20 mg a day. My Pit and remaining adrenal just don't seem to want to wake up/ I have heard others say the longer you were sick and the higher the cortisol you had the longer it takes to recover. Anyway I hope you find other peoples experiences to be helpful. I don't think people who do not have this illness understand how painful and exhausting weaning can be!
Tapering Glucocorticoids Following Surgery
By Karen Campbell
In response to the web based survey we conducted following the last newsletter, the CSRF received many requests to provide more information on tapering replacement medications and coping with the recovery process. We recognize that this process is different for each one of us, so we asked our members by email to describe their tapering process following surgery, and to describe their recovery and how they coped with the recovery process. We received a total of 91 responses, 28 of whom had adrenal tumors and 63 who had pituitary tumors. The questions asked were as follows:
1. Are you currently taking cortisol (Cortef, hydrocortisone, prednisone, dexamethasone) replacement medication?
______no _____yes (Go to question 4)
2. If you are no longer taking cortisol replacement medication, how long after your surgery were you able to discontinue replacement medication? ________months
3. If you are no longer taking cortisol replacement medication, please describe the tapering procedure you used and what tests you had done before discontinuing replacement.
4. How would you describe the recovery process?
5. What did you find helpful in coping with the recovery process?
6. If you consider yourself recovered, how long did your recovery take? ________months
While the results from this survey have not been evaluated for statistical significance and the sample size is relatively small, the general results were as follows.
28 patients responded
19 reported that they had tapered and were no longer on replacement
The range in months for tapering was from 2-50 months
Average time to taper was 19.3 mo
15 patients reported that they had "recovered"
Range for recovery was 9-50 months
Average time to "recovery" was 27 mo
Average time between stopping replacement and recovery was 5.9 months
63 patients responded
31 patients reported that they had tapered and were no longer on replacement.
Range for tapering was between 0-30 months (One response fell well outside the normal range at 108 months and was not included in the average below.)
Average time to taper was 11.2 months
30 patients reported that they had "recovered"
Range for recovery was 5-48 months (One response fell well outside the normal range at 158 mo. and was not included in the average below.)
Average time to “recovery” was 21.3 months
Average time between discontinuing replacement and "recovery" was 12 months.
It is interesting to note that the adrenal patients seemed to take longer to taper, but the time between stopping medication and "recovery" was shorter. While pituitary patients seemed to taper faster, the time after discontinuing medication was longer before "recovery". The difference in recovery between adrenal patients at 27 months and pituitary patients at 21.3 months may not be statistically significant. What follows is a discussion of the tapering process and individual responses from those participating in this survey. Recovery and coping with recovery will be discussed in the next issue of the CSRF newsletter, however we will make every effort to post the information sooner in the Articles on Coping section of our website.
The Tapering Process
As you are probably well aware, during Cushing’s the body’s normal feedback system for tightly controlling cortisol levels does not function normally. With adrenal patients, it is common for the adrenal gland without the tumor to atrophy and stop making cortisol. It is also common for the pituitary to stop making ACTH, the pituitary hormone that stimulates the adrenal to produce cortisol. Thus, when the adrenal tumor is removed, more than likely, the body cannot immediately make cortisol and needs time to recover. In the case of pituitary tumors secreting ACTH, the excess ACTH causes the adrenals to make too much cortisol. Usually, the excess of cortisol causes normal pituitary tissue to stop making ACTH. Thus, when the pituitary tumor is successfully removed, the normal pituitary tissue is usually not immediately able to secrete ACTH to stimulate production of cortisol by the adrenal glands. Again, the body needs time to recover normal function.
During this period of time, patients are placed on replacement medication. The body needs cortisol to function normally and adrenal insufficiency is extremely dangerous. You should NEVER discontinue replacement steroids without consulting your physician. Generally, following surgery, the dose of replacement steroid is gradually reduced or tapered until the body is able to make a sufficient amount of cortisol. Generally, blood tests are taken periodically for serum cortisol in the AM before the morning dose of replacement medication is taken and in some cases an ACTH stimulation test is used. Patients should be informed to watch for symptoms of adrenal insufficiency such as vomiting, diarrhea, and low blood pressure, which can lead to shock.
Generally, if too high replacement doses are used, the body will not start to produce more cortisol because it is happy with the amount that it has synthetically. Thus, to stimulate the body to make it’s own cortisol, replacement medications are often gradually decreased or tapered according to the patient’s ability to mange the aches, pains, nausea and fatigue that occur while the body gets used to lower steroid levels. Most physicians prefer to taper patients off replacement using hydrocortisone (Cortef) rather than longer acting prednisone or dexamethasone. Use of shorter acting hydrocortisone leaves the patient with low levels during a portion of the day which can assist in stimulating the body to start making it’s own cortisol. Each patient’s experience seems to be different. Some patients have extreme difficulty tapering their replacement dose while others do not.
Adrenal Patient Comments
“I would get my levels measured often, but I more expressed how “Cushing’s” I felt when I took the original high dose. I was told I needed to be patient and not “crash” my system. So, we tapered from 2 pills/day to 1.5, then to 1, all under a doctor’s supervision.” Tapered in 2 mo, recovered in 12 mo. email@example.com
“I started at 30 mg (20 am; 10 pm). I tried my first taper 2 months after surgery but it was unbearable, so my endo and I decided to take it really slowly. We tried again several months later, reducing one dosage by 5 mg. We gave my body plenty of time to fully adjust to each taper. I had periodic blood tests which did not reveal any activity in the suppressed gland. I sensed a change about 10 mos. after surgery, and a blood test confirmed activity. We continued to taper until I was down to 10 mg in the am and 5 at night, then eliminated the evening dosage, then finally the am.” Tapered in 13 mo, recovered in 13 mo.
“I went through almost three years of the 'tapering process' and at the end I was down to 2.5 mg of cortisol. My remaining adrenal gland just never 'woke up.' Finally, my doctor put me on a maintenance dose of 7.5 mg. Three years later, I feel fine. I'll be 64 in January and lead an active life.” Recovered in 42 mo. firstname.lastname@example.org
“After being released from the hospital, I was on 20 mgs of hydrocortisone. I got pushed up to 30 mgs because my staple area got infected. Then I was told to go cold turkey (!) after 1 month. I went into adrenal crisis in 5 days, got a shot of dexamethasone and then went back to 20 mgs after another week of 30 mg hydrocortisone. Then I did 20 mgs 1 day, then 15 mgs the next and alternated back and forth. I gradually tapered that way. There was really no schedule per se. I just took it down gradually over months. At eight months, I was down to 5 mgs every other day. I was told I could quit, but I could feel the difference. I stopped taking it on my own when I was able to help with moving myself to another city which was when I realized my HPA Axis had returned. I did no follow-up testing because of lack of knowledge on the part of my endo.” Tapered in 9 mo, recovered in 24 mo. email@example.com
“I was on predinsone for a year which was terrible. Then I went on 25 mgs per day of hydrocortisone. Every time I got a little ache or pain, they upped my dose and never lowered it. After 3 years plus, I found an expert endo that put me on a tapering plan. I was off in 6 months! The first step was giving up the afternoon dose. I took 20 mg in the morning only for a few weeks or until the body adjusts, then I went to 18.....then 16, 14, 12, 10. When I was on 5 I stopped. If you are on 15, it means your cortisol is getting there.” Tapered in 50 mo, recovered in 50 mo.
“The best tapering was done when I began to actually shave a little off my afternoon dose. Using a razor to cut the pills in half was too big a difference, so I began to use the razor to shave a little bit off. When I was feeling better, I would shave a little more off. This was not a perfect measurement, but it got the job done.” Tapered in 24 mo.
“About 6 months following surgery I had extreme back pain and burning skin; these pain syndromes lasted for several months. Because of this extreme body pain, my tapering had to be done in very small increments, therefore this was a long and painful process. I was able to keep going with the process because I knew the end result would be well worth the work I put in. I am describing the tapering I used after I had already tapered down to 10MG Cortef. The below schedule was using 5MG Cortef tablets:
1 tab AM - 1/2 lunch - 1/2 supper
1 tab AM - 1/2 lunch - 1/4 supper for 3 weeks
1 tab AM - 1/4 lunch - 1/4 supper for 3 weeks, then AM Cortisol and ACTH blood tests
1 tab AM - none lunch - 1/4 supper for 3 weeks
1/2 tab AM - 1/2 lunch - 1/4 supper for 3 weeks
1/2 tab AM - 1/4 lunch - 1/4 supper for 3 weeks
1/2 tab AM - none lunch - 1/4 supper for 3 weeks
1/4 tab AM - none lunch - 1/4 supper for 3 weeks
1/4 tab AM for 3 weeks, another AM Cortisol and ACTH Each taper was done for a minimum of 3 weeks, then if I felt like I was ready to move on to the next taper I did, if I still felt much pain and fatigue I would stay with that taper for another week or so before moving on to the next.” Tapered in 44 mo, recovered in 50 mo. firstname.lastname@example.org
“My endocrinologist required me to take a 16-month sabbatical from work for the tapering process and he reduced me 2.5mg at a time very gradually with constant monitoring of cortisol levels.” Tapered in 36 mo, recovered in 36 mo.
“I started with Cortef, but after about 1 1/2 months, switched to Prednisone because I was retaining a lot of water (mainly legs and feet) and it seemed to be linked to the Cortef. But after less than 2 months I switched back to Cortef because I realized I needed a drug with a shorter half life. For the first 11 months I was taking both a morning and afternoon dose. A visit to another endo led me to move to just morning dose. At 7 months I didn't take my morning dose, went in for a blood draw, had an injection of (I forgot the name of the hormone), and 45 minutes later had a second blood draw. Result - a disheartening 0.7 After a couple of months on just a morning dose a simple blood draw in the morning before taking my Cortef resulted in 7.0. I had a set back with my tapering due to high stress, lumpectomy at about 13 months. But then started taking Cymbalta to assist with joint discomfort and sleep problems. Since then, I was able to taper off. At 15 months I was only on 2.5 mg/day, so I skipped a Cortef dose, went in the next day for a blood draw. Result - 13.6 At that point, my endo recommended I go down to 1.25mg/day. No problem with that tiny amount. So after a month I started alternating days of taking 1.25 and none. After 2 weeks of that I just stopped. I have not yet taken a test to confirm this was the appropriate time to discontinue but did consult with my endo via e-mail to do so.” Tapered in 17 mo. email@example.com
“I started at 100 mg after surgery and quickly went to 80, 60, 40 and 20. Then slowly went down from there, 15, 12, 10 etc.” Tapered in 18 mo. Melissa_cottrell@sbcglobal.net
“I had monthly blood work to determine the activity of my remaining adrenal gland. I tapered approximately 10 mg per month, beginning at 60 mg. Sometimes I had to wait an additional month as withdrawal was too painful.” Tapered in 12 mo, recovered in 24 mo. firstname.lastname@example.org
“The taper was started pretty high, 300 mg of hydrocortisone, I believe. I tapered down 50 mg every 2 weeks until 50 mg, then tapered down 10 mg every 2 weeks. I tried stopping cortisol replacement after 10 mg, but I had too many aches so then I switched to dexamethasone and tapered with that 1mg down to .25mg over the remaining course of it.” Tapered in 9 mo.
Pituitary Patient Comments
“I used Cortef 40 mgs total to start....20mg am and 20mg pm.....dropping down 5mg each time of day every week or so. I would go up if I had adverse effects. Then back off again with the same schedule. This continued until I finally went down using 2mgs am then 2mgs pm... This continued until I completed the withdrawal process.” Tapered in 7, recovered in 30 mo.
“Every time I had to stop all hydrocortisone for testing, I would drop the amount I took. The blood testing was to see if my pituitary was starting to work. No specific testing was done just before stopping my hydrocortisone. Since my pituitary was starting to once again function normally, it was left to me to continually reduce it down to a point that I no longer needed replacement medication.” Tapered in 12 mo, recovered in 27 mo. email@example.com
“By the time I left the hospital (3 days after surgery) I think I was down 50%. I was taking 40 mg hydrocortisone AM and PM. I saw my endo 2 weeks after surgery and he had me knock down the PM to 20 mg, leaving me with the 40 mg in the AM. I saw him once a month for several months, each time we would lower the dose a little bit. By the time I was 5 mos. post op he started checking my ATCH (?) levels. The last time we did this he said that I was almost ready to go off the hydrocortisone, but not quite. By then he had me down to a quarter of a pill in the AM (5 mg). Several weeks after that appointment I emailed him and told him that I was going to try and stop taking that and he said OK and told me what signs of adrenal insufficiency to watch for. I didn't have any problems with that then.” Tapered in 9 mo, recovered in 48 mo. firstname.lastname@example.org
“I dropped anywhere from 2.5 mg - 5.0 mg of Cortef every 3 weeks starting with the afternoon dose until it was gone and then went to the morning dose. I really didn't have any testing done to determine if I was doing OK - just went off how I felt - now I take some only on days that I have a "low feeling".” Tapered in 30 mo, recovered in 35 mo.
“ I cut down the number of tablets every couple of days until I was taking none. I had blood work and urine cortisol tests to determine whether or not I needed further cortisol replacement therapy.” Tapered in 3 mo, recovered in 18 mo.
“I was ready to wean off Cortef 12.5mg at 1-1/2yr. post op per my ACTH stimulation test. I then saw another endo who put me on 7 mg. of prednisone and it then took another 7-1/2yrs to wean me down. I had to finally see an expert in LA to get help in weaning off. It took me 4 yrs. after seeing him to get off.” Tapered in 108 mo.
“I don't remember the names of the tests, but there were blood tests on a regular basis, and sometimes saliva tests. We kept cutting the cortisol in half and waiting a month or so and then decreasing it more.” Tapered in 12 mo, recovered in 24 mo.
“ I took 40mg after surgery, then 30mg daily for several months then 25mg (same), 15mg (same) 10mg (same), 5mg (same). ACTH measurements were done every 3 months. ” Tapered in 12 mo, recovered in 24 mo. email@example.com
“I was sent home following surgery on 20 mg hydrocortisone. My physician reduced the dose by 5 mg per month.”
Tapered in 5 mo, recovered in 5 mo. firstname.lastname@example.org
“Post-op I was taking Dexamethasone (.50mg Am, .25 mg PM). 18 days later I switched to Cortef (20mg AM, 10Mg PM); 3 months post-op I went down to 20 mg/day; 5 months post-op 10mgday; 6 months =0 mg!!!!” Tapered in 6 mo, recovered in 36 mo. email@example.com
“Taper slowly. Even then it isn't easy. I knew my system had turned on. I don't know the mechanism. I just felt different. After gradually reducing the dosage my cortisol was normal. Blood tests showed it.” Tapered in 6 mo. firstname.lastname@example.org
“I took Cortef for about a year and a half and I was only taking 20mgs for the first 2 months or so. Then I took 10mgs for a very long time after that. For the last few months I took 5mgs when I felt like I needed it. I was regularly tested for plasma ACTH and that came back to normal after about a year I think. I had one ACTH challenge after about a year and failed. Then I had another at about 18 months and did ok. That is when I went off the Cortef.” Tapered in 18 mo, recovered in 24 mo. email@example.com
What Physicians Have to Say About Tapering
We then asked several members of our Medical Advisory Board to describe the tapering process they use in their practice for both adrenal and pituitary causes of Cushing’s. We also asked how they advise their patients about recovery and what they suggest for coping with the recovery process. Again, recovery and coping will be covered in the next newsletter.
Dr. Anne Klibanski, Mass. General Hospital, Boston
“In a patient with Cushing's who has had successful removal of the tumor, either pituitary or adrenal, the patient should be hypoadrenal (adrenally insufficient). This is because the high levels of cortisol caused by the tumor will have suppressed the normal hypothalamic-pituitary-adrenal axis. To test for adrenal insufficiency after surgery while preventing symptoms of adrenal insufficiency, we give a small amount of a glucocorticoid (cortisol compound) called dexamethasone, which will not interfere with our ability to test the axis. A small amount of dexamethasone is given in the post-operative period (typically less than 1 mg/24 hours) and tests of serum, urine and late night salivary cortisol are performed. If the tumor has been completely removed, these results will be very low, and the patient said to be "cured" or "in remission". If the levels are not very low, the tumor is likely still there, or, a longer time period may be needed to assess the person's axis, because in rare cases, the cortisol levels fall more slowly. If the cortisol level is very low, the patient is adrenally insufficient and must take a small amount of a glucocorticoid (such as prednisone or hydrocortisone) every day to be healthy. The goal is to give enough cortisol replacement so that the patient does not have adrenal insufficiency, but not so much that the system is suppressed. There are no biochemical tests to indicate the best dose for an individual patient; this is determined by talking with and examining the patient.
In the case of an adrenal tumor, in which one adrenal gland is often removed, we expect the normal axis to recover, because the other adrenal gland should eventually be able to produce cortisol. In the case of a pituitary tumor removal, the normal axis will usually recover, but if the normal gland was permanently damaged by the surgery or the tumor itself, adrenal insufficiency may be permanent and glucocorticoid replacement may be life-long. This is not the usual case when surgery is performed by an expert pituitary surgeon, but can happen. It takes many months for the normal axis to recover; often up to a year, and rarely even longer. If testing after a few months still shows very low cortisols, we wait a number of months before re-testing to see whether the normal axis has recovered.”
Dr. Andre Lacroix, Univ. of Montreal, Montreal, Canada
“We use only hydrocortisone post-op of pituitary or adrenal CS if plasma cortisol is low following pituitary surgery or adrenal surgery. If CS was severe pre-op, we usually decrease the post op solu-cortef from 50 mg IV every 8 hours down to oral hydrocortisone 40 mg after 2-3 days post-op (20-10-10). If CS was less severe, patients are discharged with 30 mg (10-10-10). Patients are seen after 1-2 weeks for determination of symptoms. If withdrawal symptoms are severe with a lot of joint pain, nausea etc, it may be necessary to increase dose slightly. Patients are seen for 2-3 visits at weeks 4, 8, and 12 and the dose is progressively decreased by 5 mg (20-10-5; 20-5-5; 20-5-0). As soon as possible the Cortef dose is decreased to 20 mg in morning only which then facilitates better recovery of ACTH-cortisol axis. At that time (20 mg morning only), we see patients every 2-3 months with morning fasting cortisol prior to hydrocortisone dose. When cortisol is above 200 nmol/L (7.25 ug/dL), we then test with ACTH 1 mcg dose and stop replacement as soon as plasma cortisol increases above 500 nmol/L (18.12 ug/dL) post ACTH. If morning level is > 200 and the response close but short of 500 nmol/L, Cortef is decreased to 10 mg in the morning until a normal response is attained ie > 500 nmol/L post cortrosyn. Patients maintain a medical bracelet and instructions to increase Cortef in case of stress or illness until full recovery is restored.”
Dr. Lynnette Nieman, NIH, Bethesda MD
“I use same regimen for all types of CS. Initiate replacement after surgery at a replacement dose, not supraphysiologic, 12 - 15 mg/M2 of hydrocortisone. If patients are extremely symptomatic on that, I will go up 5 mg --usually not more-- on the daily dose. I taper based on weight loss only. For testing, measure cortisol or perform ACTH stim test beginning at 6 months, every three months. If baseline or stimulated cortisol is 18 ug/dL or more, stop steroids altogether. If values are close, I might re-test earlier, or if the basal cortisol is 10 or more, I might do an ACTH stim test sooner.”
Dr. David Schteingart, Univ. of Michigan, Ann Arbor
“Following surgery and within 24 hours post TSS we hold hydrocortisone beginning at 6 pm. We retest patient's ACTH and cortisol levels beginning the next morning. If levels are completely suppressed, we discharge patients on 25-35 mg of hydrocortisone daily, depending on how high the pre-op cortisol levels were. If they were very high, we start with 35 mg, since patients may be more at risk for withdrawal symptoms on the lower dose. We see patients in clinic every 2 months. If they are stable, we begin a slow taper over a 4 month period to a level of 20 (10-5-5) mg/day. We may get a urine free cortisol while on that dose, aiming at a UFC in the mid normal range. We find some people have supra-physiological levels on an otherwise average maintenance and this delays recovery of HPA axis function. Beginning 6 months into the taper, we check ACTH and cortisol levels first thing in the am before taking the first dose of hydrocortisone. If the ACTH levels are rising, we continue the taper. Most of our patients need 9-12 months to recover HPA function. In one case of recovery from a cortisol-secreting adrenal adenoma it took 10 years!! before patient was able to discontinue replacement.”
Dr. Mary Lee Vance, Univ. of Virginia, Charlottlesville
“There is NO one best way for every patient; tapering of steroids is like a “tailor made suit” – it has to be done for each patient. All patients are different regarding their symptoms when reducing the steroid dose and the time required to finally discontinue steroid replacement (presuming that normal pituitary tissue was left after pituitary surgery). Another important point: there is no way to know how long a patient will require steroid replacement (waiting for the normal pituitary ACTH cells to recover) – it may take a couple of months to 6-12 months, everyone is different.
Tapering of steroid replacement
Pituitary Cushing’s: If the patient has had a decrease in serum cortisol after surgery (we don’t give steroids at the time of surgery [some centers do], so we know before the patient leaves the hospital if he/she needs cortisol replacement [successful surgery]). If the post operative cortisol is low, the patient is sent home on a decreasing hydrocortisone regimen (hydrocortisone, 40 mg on awakening, 20 mg at 6 pm – for 1-2 weeks, then he/she reduces the dose to 20 mg on awakening, 10 mg at 6 pm). The patient is asked to stop the hydrocortisone for 2 days before the 8 week post operative clinic visit and the serum cortisol and ACTH levels are measured. If the cortisol and ACTH levels are normal, hydrocortisone is discontinued. (In my opinion, a low cortisol and ACTH level 8 weeks after surgery is a very good thing, as it indicates continued remission). If the cortisol and ACTH are low, the hydrocortisone dose is reduced to 15 mg on awakening and 5 mg at 6 pm (some patients do well with 10 mg on awakening and 5 mg at 6 pm; it all depends on how the patient feels). Since most of my patients are from long distances, I send them a letter advising re-testing in about 2 months (off hydrocortisone for 2 days) with their local Endocrinologist. If cortisol and ACTH are again low, repeat the cycle 2 months later and again 2 months later if cortisol and ACTH levels are low. Again, every patient is different; there is no certain “formula” that applies to every patient.
Adrenal Cushing’s: We give hydrocortisone (intravenously) during the operation and during the post-op hospitalization (we expect the pituitary gland to be suppressed, no ACTH production). The patient is discharged on a tapering dose of oral hydrocortisone, same as for pituitary patients. The postoperative evaluation is the same as for pituitary patients (stop hydrocortisone for 2 days, measure blood cortisol and ACTH levels). Most of my adrenal Cushing’s patients have recovery of pituitary/adrenal function within 3 to 6 months after adrenal surgery, but I have one patient who has not recovered after 4 years (she had 2 successful pregnancies – a mystery to me as to why her cortisol and ACTH levels remain low despite a stimulation test).”
While the tapering process is different for individual patients and different physicians suggest different tapering processes, the goal is very clear; decrease the replacement dose as the patient can tolerate with the goal of eventually discontinuing the medication completely. Recovery will be covered in the next newsletter, but a few brief suggestions include, recognize that you didn’t get sick overnight, so time will be required to recover, exercise patience, patience and more patience, be kind and gentle to yourself, and do not give up hope. Life without Cushing’s is so much better than life with Cushing’s. Others have survived, you can too!