ORGANIZATION MOXIE: Where the Rubber Meets the Road

Hi everyone.

The wean is slow but going okay. I am 5 weeks post op today. My dose of hydrocortisone is 15 mg @ 8 am and 5 mg @ 3 pm. I see my neurosurgeon and endocrinologist for a follow up next Thursday. I have been keeping track of my doses and symptoms in a spreadsheet. When I get time, I will post it here, in case it will be helpful for anyone to see how my doctors and I handled it. If it never appears, feel free to remind me!

Also, I wanted to let you all know that I have been getting myself organized lately. Nothing like finally getting papers filed after over a year! (not that I had a good excuse or anything...). I am trying to get my home office paperwork filed electronically, and it is a huge but worthwhile undertaking. I am not finished yet, and I have been working on it for a week. Wish me luck!

So, along with this new found need to organize finally, I want to be better about answering questions that you readers may have. I wanted to let you know that I am now in a better position to answer any emails that you send me. Before, I was completely overwhelmed with Cushing's and my sweet baby. Now after surgery, I feel I have things under control, well, more than I did. I have an iPhone (greatest invention ever--it's a computer in the palm of your hand!), and this allows me to check email every few days without getting on my laptop. With a baby in my arms most of the day, this is a wonderful addition to my toolbox.

As always, if you want to post a comment, feel free to do so. I welcome and encourage that. Please be forewarned that I like to publish your stories to show others how this disease sneaks up on us.

If you have a specific question, please ask it in the comments section, include your email address, and I will be sure to reply to you via email. If it is ok, I would like to post all incoming emails to my blog in the comments section, and if you include your email address, I will just cut and paste your message and post it under comments myself. That way, I will not post your email address publically.

For direct access, my email address is moxiemelissa then at symbol gmail.com (I am trying to avoid spam by not posting the email addy here).

Anyway, I better scoot. Take care. I hope everyone is doing ok.

~Moxie Melissa

NAPS: The Search for the Ultimate Afternoon Delight

I started napping for the first time since my pituitary surgery four weeks ago. It started this past Tuesday, when I dropped my dose of hydrocortisone from 25/10 to 20/10. With my sweet Elena at my mom's house, I stayed in bed all morning, thanks to meals and meds from my work-at-home-today-because-he's-working-Saturday-night hubbie.

I love a good nap. I hope you get a good one, too.

~MM

Thanks to the MaryO and her Cushing's & Cancer blog for sharing this entry originally posted on the MS Brass and Ivory blog. This made me smile.

The steroid taper finished up this past Sunday, but instead of finding some new energy, I've been caught in a fog of fatigue. Without too much choice in the matter, I've drifted off to the land of comatose and nod for hours during the past three days. As I know many of you have been in the same place far more often than either of us would like, I thought you might enjoy the following poem taken from Falling Up by Shel Silverstein, a well-loved book which sits in the waiting room of my music studio.

The Nap Taker
No--I did not take a nap--
The nap--took--me
Off the bed and out the window
Far beyond the sea,
To a land where sleepy heads
Read only comic books
And lock their naps in iron safes
So that they can't get took.

And soon as I came to that land,
I also came to grief.
The people pointed at me, shouting,
"Where's the nap, you thief?"
They took me to the courthouse.
The judge put on his cap.
He said, "My child, you are on trial
For taking someone's nap.

"Yes, all you selfish children,
You think just of yourselves
And don't care if the nap you take
Belongs to someone else.
It happens that the nap you took
Without a thought or care
Belongs to Bonnie Bowlingbrook,
Who's sitting' cryin' there.

"She hasn't slept in quite some time--
Just see her eyelids flap.
She's tired and drowsy--cranky too,
'Cause guess who took her nap?"
The jury cried, "You're guilty, yes,
You're guilty as can be,
But just return the nap you took
And we might set you free."

"I did not take that nap," I cried.
"I give my solemn vow,
And if I took it by mistake
I do not have it now."
"Oh fiddle-fudge," cried out the judge,
"Your record looks quite sour.
Last night I see you stole a kiss,
Last week you took a shower,

"You beat your eggs, you've whipped your cream,
At work you punched the clock,
You've even killed an hour or two,
We've heard you darn your socks,
We know you shot a basketball,
You've stolen second base,
And we can see you're guilty
From the sleep that's on your face.

Go lie down on your blanket now
And cry your guilty tears.
I sentence you to one long nap
For ninety million years.
And when the other children see
This nap that never ends,
No child will ever dare to take
Somebody's nap again.

TAPERING YOUR DOSE OF CORTISOL REPLACEMENT POST OP

I have not actually done the wean itself yet. It comes after pituitary surgery!!

I found this helpful in explaining the overall hormone replacement process, and why it is "common" for Cushies to end up in the ER after pituitary surgery. I cannot attest to the tapering schedule and amounts of hydrocortisone to take and when. Please consult with your doctor. ~Melissa

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TAPERING YOUR DOSE OF CORTISOL REPLACEMENT POST OP: From Oregon Health and Science University

The experience of cortisol withdrawal during the first several months after being cured of Cushing's can be very unpleasant. During this period, cortisol must be given back to the patient and then tapered off over time. Classic symptoms of this withdrawal process include fatigue, aching, and depression. Abdominal pain, nausea and vomiting, and dizziness are indicators that the cortisol withdrawal is occurring too quickly (adrenal insufficiency is occurring) and the hydrocortisone taper should be slowed.

There are two main phases that a cured patient will go through after surgery. The first is the cortisol withdrawal phase. The features of Cushing's disease are due to cortisol excess and the body reacts to having the cortisol withdrawn. To make the process tolerable (and safe), large doses of glucocorticoids are given to the postoperative patient and then tapered over a month or so as symptoms allow. This can be an awful experience for the patient marked by fatigue, depression, and body aches. Headache may be present but if severe (especially if accompanied by nausea and dizziness) may indicate that the taper is happening to quickly. After the initial withdrawal phase, the second phase is marked by the temporary need for continued glucocorticoid replacement (typically 20 mg hydrocortisone or 5 mg prednisone) until the hypothalamic/pituitary/adrenal (HPA) axis awakens. This phase may last many months and may last over a year. Until this axis awakens, the patient is adrenally insufficient and should be treated accordingly.

Assessment of Cure
Just as the initial diagnosis of Cushing's disease can be difficult to make, "proving" someone has been cured of Cushing's can be very challenging as well. One early indicator of biochemical cure is the measurement of very low (or undetectable) post-operative morning cortisol levels (typically measured 72 hours after surgery). In this post-operative setting, morning cortisol levels are typically low because normal ACTH producing cells in the pituitary have been suppressed by elevated serum cortisol levels. Therefore, the removal of the ACTH secreting tumor leaves no source of ACTH, the adrenal glands are no longer stimulated and cortisol levels plummet. It is important to note that as high as 30% of patients with long term cure of cushing's disease do not have a history of undetectable 72 hour post-operative serum cortisol levels. Therefore, if 72-hour post-operative cortisols are not below 5 ug/dl, further evidence should be obtained to confirm the presence or absence of cure.

After a patient has been tapered off replacement glucocorticoids post-operatively, it is important to demonstrate the normalization of the tests that were initially used to diagnose Cushing's disease. For example if a patient had a significantly elevated 24-hour UFC pre-operatively, it should normalize if a cure was obtained. Other evidence of cure is the dramatic resolution of the features of Cushing's disease. For example, many patients note a dramatic weight reduction in the first few months after surgery. By contrast, even in the context of a biochemical cure, features may resolve slowly. Even in the context of a biochemical cure, the symptoms of fatigue and depression can persist for many months since they are also the symptoms of cortisol withdrawal. It is important to note that even with an incomplete cure (i.e., not all the pituitary tumor was removed), many symptoms will begin to resolve at first but they usually return over time.

In general, when a cure is obtained, most of the features of Cushing's disease will reverse over time. Some of these changes can be dramatic. Some may take years. Of note, the need for medical treatment for high blood pressure and diabetes should be evaluated closely and will likely need to be tapered.

Finally, even in the hands of an excellent neurosurgeon, there is a recurrence rate of about 10% over time for Cushing's disease. Therefore, even in the context of a "biochemical cure", Cushing's patients should be monitored by history, physical, and biochemistry over time for possible recurrence of their disease. Work up and treatment are similar to those done at initial presentation.

Long-term Course and Potential Complications

Cured Cushing's are typically delighted with the resolution of most of their Cushing's symptoms. This can, however, take from months to even years to fully happen. As stated above, recurrence does occur and should be watched for by the patient and their clinician. Associated diseases such as diabetes mellitus, hypertension, and osteoporosis should be followed (and treated if necessary) although each may resolve fully over time as well.


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TAPERING YOUR DOSE OF CORTISOL REPLACEMENT POST OP, Cushing's Research and Support Foundation

Question: I have been trying to taper my replacement dose of hydrocortisone following pituitary surgery, and I have ended up in the Emergency Room several times due to adrenal insufficiency. I have been told that for my adrenal gland to start producing cortisol and working properly again, I need to be this low. Is that true? What tapering protocol would you recommend and what tests should be performed during the tapering phase?

Answer: These are very good questions. Tapering of hydrocortisone following the removal of an ACTH-producing pituitary tumor, bilateral adrenalectomy or a single Cortisol secreting adrenal tumor results in profound hypothalamic pituitary adrenal suppression, especially when it is successful. The reawakening of this axis may take six to nine months, sometimes longer.

In the interim, patients need to be replaced with glucocorticoid therapy. There is an additional confounding problem and that is when you have Cushing's, your body gets used to higher doses of glucocorticoids; therefore, such high doses need to be continued above replacement doses immediately after surgery and then slowly tapered.

Right after surgery we replace with Hydrocortisone 60 to 80 mg a day for two weeks and start a taper, diminishing 10 to 20 mg every 10 to 14 days, until you are down to physiologic, which is in the neighborhood of 15 to 20 mg per day.

We like to do a Cortrosyn stimulation test every two months to plot the hypothalamic pituitary adrenal recovery. Morning plasma cortisol before taking oral cortisone may also be useful, but we prefer the cortrosyn test. Once the Cortrosyn stimulation test is in the normal range, then Hydrocortisone can be safely discontinued, or tapered to a lower dose, then discontinued.

We do not suggest longer acting steroids, such as Prednisone, which might further prolong pituitary adrenal suppression. The use of Hydrocortisone alone is what is suggested immediately after successful surgery. There is not only a need for Glucocorticoid replacement to avoid adrenal insufficiency, there is a withdrawal from the high endogenous levels. When you withdraw from steroids, even though you are in the normal range for replacement, you will have symptoms of steroid withdrawal which include fatigue, depression, and muscle and joint aching. This is why endocrinologists will tell you that you will feel worse from steroid withdrawal after successful surgery than you felt with the Cushing's syndrome. The steroid withdrawal symptoms can last up to two years and they are again, fatigue, depression, and aching.

There is no good evidence that taking less steroid, or no steroid, accelerates the hypothalamic pituitary adrenal axis recovery. Certainly you cannot use high doses without continuing to suppress the pituitary adrenal axis, but in general, completely stopping steroids is not suggested, since adrenal insufficiency would be predicted under those sets of circumstances and also aggravation of severe withdrawal symptomatology.

In summary, it is important to treat adrenal insufficiency symptoms of lightheadedness and nausea, and also distinguish that from steroid withdrawal symptoms, which are fatigue, depression, and achiness, even though you might be protected from adrenal insufficiency.