Day 17 of the Cushing's Awareness Challenge: Adrenalectomy Improves Quality of Life for Cushing's Patients Although It May Take Years

Although adrenalectomies are only a first-line treatment for those with ACTH-independent tumors in Cushing's Syndrome (ectopic and adrenal tumors, benign and malignant), they are also often a treatment for those with Cushing's Disease when pituitary surgery fails to totally remove the source of excess ACTH.

Hypercortisolemia, the result of excess ACTH from the pituitary or from overproduction in ectopic or adrenal tumors, is very debilitating for those who suffer from CS/CD. According to the authors, "Untreated Cushing’s syndrome can cause significant physical and mental morbidity and mortality, with a mortality rate in untreated that is 4 times greater than the baseline population."

This study looked at the outcomes of 60 patients who underwent either a unilateral (53%) or bilateral adrenalectomy (47%) to treat their Cushing's. Except for one patient, all adrenalectomies were done laproscopically. The median follow-up time was 41.4 months. At that median time, 9 patients had died. 4 patients had died from the progression of malignant disease, 2 from unknown causes, 1 from pulmonary embolus 2 months after surgery, and 2 five months post-op from severe complications due to pituitary (ACTH-dependent) Cushing's Disease.

Around 75% of the common physical signs/symptoms of Cushing's resolved after surgery, but the central obesity only had a resolution rate of 57%. Diabetes was cured in 79%, hypertension "improved dramatically or was cured in 67%."

Symptoms took anywhere from a few weeks to 4 years to resolve with most of the physical changes resolving in a mean of 7-9 months. There was a great variability with no good predictor of this variability.

The remark that I find so telling is this:

In fact, we had several patients with ‘‘subclinical’’ Cushing’s who took over 2 years for their symptoms to resolve.

Too often those of us who suffer with Cushing's are told to wait until the disease "gets worse" before being diagnosed/treated.

The authors point out the complication rates which are typically higher for Cushing's patients than for others who undergo adrenalectomies for whatever reason. Immunosuppression leads to greater risk of infection. Addisonian crisis is another prevalent complication. However, overall they conclude that adrenalectomies are a safe and effective treatment option, but both physicians and patients must not expect overnight improvements. This is another case of where slow and steady wins the race.



R SIPPEL, D ELARAJ, E KEBEBEW, S LINDSAY, J TYRRELL, Q DUH (2008). Waiting for change: Symptom resolution after adrenalectomy for Cushing's syndrome Surgery, 144 (6), 1054-1061 DOI: 10.1016/j.surg.2008.08.024

My BLAnniversary

....or my "ReBirthday" as my friend, Nancy, says, was a few days ago on June 16th.  It has been a year since I had both of my adrenal glands removed to control my Cushing's Disease.

How am I?  Perhaps I'll give you a clue by saying I've been too busy to write this post.  In fact, I'm having trouble finding time to blog at all!  So, I am good.  No, I am great!!  I will always have to deal with some of the ravages of Cushing's Disease, but in comparison to what life was like prior to my BLA (bilateral adrenalectomy), I'm SUPERTASTIC!!  

Some of the good things which have happened:

• I now go up steps most of the time without even thinking about it.  (Going down is still a problem due to a former broken ankle and a bad knee on the same side.)
• I've lost almost 80 pounds without even trying.
• Instead of parking in handicapped parking and using a scooter in stores, I can now park in Timbuktoo and walk all over a store.  Wait...I can even tag along with my daughters and walk through multiple parking lots and multiple stores.
• I have great hopes now of living to be a decent mother and grandmother.  Shoot, maybe even awesome sometimes!  (My first grandchild is due in July!)
• I don't mind traveling/driving all by myself and do it often.  I've made more trips to see my girls (and hubby/boyfriend) in the past few months than I have in the previous 8 years. 
• My mental state is beyond awesome.
• I find myself smiling and singing all the time.
• I'm umpteen sizes smaller.  (The bad part is all the sagging skin and eyelids.  Maybe that can be fixed in the future.)  I still have quite a bit of weight to lose.
• I don't dread things anymore. I used to dread getting up, walking, and so much more.
• I can see my ears when I look into the mirror.
• I have skinny ankles. Pretty shoes fit again. 
• I sleep all night, every night.  I go to bed at a normal hour and get up at a normal hour.

I'm sure there are a zillion other things I can write, and I'll think of them later.  There is so much improvement and I notice something every day!

Are there any "cons"?  No, not in my book.  I wouldn't trade my BLA for anything!  Sure, I have to take medication every day to live, but I get to control how much "cortisol" my body gets instead of it controlling me.  I have been fortunate and haven't had a crisis at all.  I haven't had one visit to the emergency department anywhere.   Sure, infections throw me into insufficiency pretty quickly, but the high side of that is I know I have an infection way before most folks would. 

They say a picture is worth a thousand words. I'm going to let these pictures say the rest for me about the cyclic nature of my Cushing's Disease and how long I had it:

Don't I look like a grandma in that last one??? ;)  I don't know what I was thinking with some of those hair styles.

Read more about episodic/cyclic Cushing's: 
High Prevalence of Normal Tests Assessing Hypercortisolism in Subjects with Mild and Episodic Cushing ’ s Syndrome Suggests that the Paradigm for Diagnosis and Exclusion of Cushing ’ s Syndrome Requires Multiple Testing

Postlude:

Thank you, Dr. M. for finally realizing in 2005 that what I had was endocrine and for sticking with me.  Thank you, Dr. Friedman, for believing me and listening to me, for making me a partner in my health, and for testing me.  You saved my life.  Thank you, Dr. Chiang, for trusting Dr. Friedman and for being the most caring doctor/surgeon I've ever met.  And most of all, thank you Mary O'Connor, for all you do to spread awareness of Cushing's Disease/Syndrome.  You helped me find my life again.  I love you.   I hope all of you see this so you can see what a wonderous work you do.

This would not be complete without thanking my family. My daughters have been my lifeline.  They took two weeks out of their lives and lost vacation time just to take me to surgery and stay with me.  Prior to that, they were here with me when I needed them, helped me do so many things, went with me on trips to see Dr. F, and so much more.  I love you dearly, my daughters, and love the women you are. 

My parents took care of me after surgery, finding any treat they could, cooking anything they could to help me find something I could eat.  They took me for labwork, helped me find DHEA, went to the store at odd hours to get me some gatorade, and so much more.  I love you, Mother and Dad.  And I thank you.

Thanks to all my Cushie folks who support each other.  I'm sorry you have Cushing's, too, but I'm proud to have met you, shared the triumphs and the pain with you, laughed and cried with you.  Hugs.

Blogging his BLA, Steve shares his experience...

"We are the orphans. We are the zebras in a world of horses. The ones who have a rare disease many have never heard of. We have no famous speakers to bring life to our voices. We have no sports stars to put a face on Cushing’s. We have no pink ribbons. We have no simple tests to tell us if we have it. If no one cures us, our lives will be shortened at best and ended at worst." --Steve Owens on "Should Have Seen It"

Steve is a Cushing's survivor who has already had surgery to remove a pituitary adenoma. He still has full blown Cushing's, and is going to have a bilateral adrenalectomy (BLA) on Friday, January 30. He has chosen to blog about his surgery and you can read all about it at his blog "Should Have Seen It".

Steve also blogs at Thankful for the Journey and Herding Zebras.

Watch as Steve shares his experience, good and bad, happy and sad. We are pulling for you, Steve!!

Adrenalectomy improves quality of life for Cushing's patients although it may take years

Although adrenalectomies are only a first-line treatment for those with ACTH-independent tumors in Cushing's Syndrome (ectopic and adrenal tumors, benign and malignant), they are also often a treatment for those with Cushing's Disease when pituitary surgery fails to totally remove the source of excess ACTH.

Hypercortisolemia, the result of excess ACTH from the pituitary or from overproduction in ectopic or adrenal tumors, is very debilitating for those who suffer from CS/CD. According to the authors, "Untreated Cushing’s syndrome can cause significant physical and mental morbidity and mortality, with a mortality rate in untreated that is 4 times greater than the baseline population."

This study looked at the outcomes of 60 patients who underwent either a unilateral (53%) or bilateral adrenalectomy (47%) to treat their Cushing's. Except for one patient, all adrenalectomies were done laproscopically. The median follow-up time was 41.4 months. At that median time, 9 patients had died. 4 patients had died from the progression of malignant disease, 2 from unknown causes, 1 from pulmonary embolus 2 months after surgery, and 2 five months post-op from severe complications due to pituitary (ACTH-dependent) Cushing's Disease.

Around 75% of the common physical signs/symptoms of Cushing's resolved after surgery, but the central obesity only had a resolution rate of 57%. Diabetes was cured in 79%, hypertension "improved dramatically or was cured in 67%."

Symptoms took anywhere from a few weeks to 4 years to resolve with most of the physical changes resolving in a mean of 7-9 months. There was a great variability with no good predictor of this variability.

The remark that I find so telling is this:

In fact, we had several patients with ‘‘subclinical’’ Cushing’s who took over 2 years for their symptoms to resolve.

Too often those of us who suffer with Cushing's are told to wait until the disease "gets worse" before being diagnosed/treated.

The authors point out the complication rates which are typically higher for Cushing's patients than for others who undergo adrenalectomies for whatever reason. Immunosuppression leads to greater risk of infection. Addisonian crisis is another prevalent complication. However, overall they conclude that adrenalectomies are a safe and effective treatment option, but both physicians and patients must not expect overnight improvements. This is another case of where slow and steady wins the race.



R SIPPEL, D ELARAJ, E KEBEBEW, S LINDSAY, J TYRRELL, Q DUH (2008). Waiting for change: Symptom resolution after adrenalectomy for Cushing's syndrome Surgery, 144 (6), 1054-1061 DOI: 10.1016/j.surg.2008.08.024

One Good Thing - by Steve Owens

.....

I have a dear friend, Steve, who is suffering with Cushing's. I met Steve first online at cushings-help.com's message boards over 2 years ago where he was seeking help for a known adrenal tumor. He was passed around to multiple specialists and no one would do anything.

Steve was suffering mightily from the symptoms of Cushing's, yet no one would even test him for that. He persisted and pursued with two Cushing's specialists all the way across the country and was eventually diagnosed with ACTH-dependent Cushing's due to pituitary tumors. (Since many adrenal tumors are due to high ACTH stimulating the adrenals, it was hoped his would go away with the remission of the high ACTH.) He was referred by one for surgery which was done by a top-notch neurosurgeon at M. D. Anderson. Two tumors were removed, one from his pituitary and one from the sella floor.

Steve was not cured. The hopeful demise of the adrenal tumor did not happen, and he is now facing a bilateral adrenalecomy. It will happen next month. In the meantime, he as been on ketoconazole.

Not only does Steve face this after years of his body failing him due to the high ACTH, but his younger daughter is being tested for Cushing's, too. I've met Steve's family in person, and they are as dear as they come. His wife and girls are so supportive of him, and he continues to support others as much as he can in his fight for his life.

All of the above was simply to preface the story below. Steve wrote it and gave me permission to post it. (Love ya, my friend. I know how difficult this was.)

~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

One Good Thing

If there was ever any hope it is the hope that someday Cushing's will be easier to diagnose and to treat. That doctors will recognize the symptoms and tests are developed that lead from diagnosis to a cure in weeks, not years. If there is one thing I could want to come of all this, it is that. Not just for me, but for all of you, and especially my daughter. It is the hardest thing in the world looking into her eyes. I just want one good thing to come of all this.

These past few weeks have been filled with a lot of emotions, feelings, and just plain being scared. The closer the BLA gets, the more I don't like it. I have no extra spoons to spare to try and conquer the fear, because I am using them to get through each day. The days have been filled with doctors visits, testing for me and Brooke, and work. Nothing out of the ordinary, other than having an infection we cant seem to clear up.

It was at one doctor visit, with Doctor VJ, my family doc, that is the reason of my story. You see, I haven't seen her in a year. My weekly and bi-weekly visits have all been to endo's who are taking care of everything. Well, since they were out of town, I needed to see my family doctor. This is the doctor who wrote tests and she didn't know why, other than we asked for them. She couldn't read results because Cushing's isn't so black and white. This is the doctor who looked at me and knew I had Cushing's, once she read about the physical and not so physical symptoms. This is the doctor who told us my daughter has Cushing's, like her father. This is the doctor who scoured the medical books, talked to other doctors, and researched the internet to learn what she could to help her patients.

We would learn that Dr VJ would have a new patient come to her, one she suspected had Cushing's. Because the doctor took the time to care about me, she would notice the signs and symptoms and be able to run some tests. Because she was able to see a patient that had Cushing's in the past, she was able to see it in her new one. She was able to send her to my endo because she remembered she was helping us and others. This new patient is on the road to recovery already. She is going to be alright.

This made me stop and think. I found this doctor because of the goodwill of a lawyer. This lawyer spent 10's of thousands of dollars of his own money helping me fight and beat the insurance company. This is money he doesnt want back and will not take from us. He did it out of the goodness of his heart. His words were that "there are some things that are so fundamentally wrong in the world and I am just happy that I can help you right one of them." When our family doctor gave up on us, like so many others, he sent us to Dr VJ's office. From there, the doctor and our family grew into a team to help me get better.

I feel so humbled to meet people like these. There are good people in the world. I wanted to give credit to where it is due as well. It would be easy to say that the doctors learned because I did this....or I did that...and that would NOT be true. I know in my heart that I was led to people who could help me at the time I needed it most because I believed and we prayed. I feel humbled that God put me in these doctor's offices so that I could be an instrument of learning.

I believe that every thing we say or do can be remembered, for good or bad, by those around us. Sure, we remember the big things of our lives, but I have so many vibrant memories of the little things. It isn't the big presents I remember most and hold on to dearly. It is the small random acts of kindness that I cherish the most. Everyday, we have the ability to leave a mark on someone's life, even in the smallest of ways. None of us know God's will, but I believe that in that moment of time, when I was sick and out of options, He put me where He wanted me to be and that was the that doctor's office. I had never thought of it that way. I had ALWAYS prayed that something good would come from this to help the next person. If something good was going to come from it all, it was going to come from the powers of up above, not through my works or anything I could do.

My prayer was answered. I know that something good has come from this all, even if it has helped one single person. For that, I am thankful for my blessings. I am thankful for the brief clarity of mind to write it all down.

~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

We are the champions in this race......

Carrin has had surgery twice to remove a pituitary adenoma. She just recently found out she again has tumor growing on her pituitary and still has Cushing's. She was not surprised, but obviously a bit sad. However, when she shared her thoughts with the Cushie community at cushings-help.com, they were not negative at all. They were inspiring to me and I wanted to share them with you (with her permission):

I am now at a point where I never thought I'd be. I am content with where my life has taken me and although the road has been long and sometimes difficult I wouldn't change a thing. I have been changed by all the people along the way and I am learning how to live my life. Sure maybe it isn't the plan that I had originally, but that is what makes it an adventure.

We have an advantage over people who have never been ill. We move slower to enable us to see more around us, enjoy it, learn from it and pass that knowledge on. Remember Aesop's tortoise and the hare? The hare moved so quickly that he decided he could take a nap. In the meantime the tortoise slowly and steadily won the race. What do you think the hare enjoyed in his hurried need to finish first? I always imagine this tortoise who Aesop says was ridiculed for his short feet and slow pace -- I imagine him enjoying the feel of the grass beneath his feet, the smells, the sounds and the view!

Cindy* your lighthouse post really made me happy. I imagined the hundreds of tourist and hikers who begrudingly climed the steps of that lighthouse just to finish. Just to do it. You on the other hand understood the value of each of those steps, and relished in a victory.

This disease changes us, during this illness we become so angry and upset that we begin to measure our worth on the amount of steps we take in the day... we are just like the hare. We want to finish first. Now I realize that it isn't the amount of steps I take in the day... it truly is the quality of those steps. I'm slower and weaker than most, but I do have the power to win the race. Slow and Steady. Somedays I may only get out of bed a couple times, but from my bed I am able to dream. On good days I may walk my kids into their classroom and be a huge part of their world. I can hold the hand of someone who is hurting. I can be a friend. I can bake a cake for my elderly neighbor. I can pray. I used to see those things as limitations... I can only.... I can only.... You see, just because people can doesn't mean they do. We are those people who CAN do.

We are the champions in this race.

*Cindy recently had a bilateral adrenalectomy after a failed surgery to remove all tumor tissue from her pituitary. She shared her recovery with us, including pictures of a hike made to a lighthouse while on vacation. It was very inspiring.

Gals, I just want you to know that you inspire me. Thank you so much for sharing.