Adrenal Crisis & BLA

Take a listen to JenS as she describes her Cushing's journey and adrenal crisis.

*Thank you to MaryO, founder of www.cushings-help.org, for creating this series of podcasts.

Looking back at the path you chose

In case you haven't noticed, helping other Cushing's patients wade through the cortisol quagmire is kinda a big deal for me. I choose to spend my free time doing it. Why?

I don't like seeing others struggle. 

I don't like that Cushing's is ruining Cushies' lives. 

I don't like how poorly doctors treat us. 

I detest inefficiency.

So I do my best to take time and answer questions from blog readers and facebook friends.  Tonight, the post was about ketoconazole. The Cushie had gone to the emergency room twice for adrenal insufficiency and adrenal crisis while taking ketoconazole. 

I remembered that I too took ketoconazole. My memory isn't what it used to be, and as such, I set out to search for the word keto on this blog, for so much time has elapsed that my Cushie brain can't hold the details any longer. Fair enough. 

I found the link I wanted (Keto: It Was Fun While It Lasted), but I found the post that proceeded that one by just three days called New Chapter of my Life dated November 3, 2013.  I shared the link for you to review if you are so inclined.

I read through this lost post wondering what was the new chapter of my life. I don't remember any new chapter of my life?! I was floored with emotion as I read my own words recounting the story of my decision to proceed with a bilateral adrenalectomy, which doctors often dissuade us from considering because BLA is the last resort treatment for Cushing's. 

The phrase, "I did everything the medical community asked of me" really hit home. Because don't we all? Aren't Cushies pushed beyond the point we can handle emotionally, mentally, and physically every single day and somehow manage to fight on until the day we reach remission? Does anyone remember my explanation for how statistically rare it is to have Cushing's and yet, we do? Cushies have hit the unlucky jackpot? for Cushing's.

BLA is not easy. It has taken many, many days to get to know my body and what it needs. I do stand tall in the explanation that I made to myself at that time, as it is still one that stands up to reason and emotion. It stands the test of time. 

~*~*~*~*~*~*~

I will post one day soon about the improvements I've experienced since BLA. I will foreshadow that it is difficult to parse out what long term effects are from Cushing's and high cortisol and what I can blame on rheumatoid arthritis. That's for another day.

See you soon, Cushies.

Three year BLAnniversary

It's my BLAnniversary! Three years ago, I had my malfunctioning adrenal glands removed in an effort to cure myself of Cushing's disease. I underwent two unsuccessful pituitary surgeries, medication therapy with ketoconazole, a drug that lowers cortisol, for 15 months, before deciding to undergo what many doctors call "a surgery of last resort." Not exactly the words that make a patient feel warm and fuzzy, yet I moved forward like many of Cushing's friends did before me and after me, and I pursued a life free of excess cortisol. Big risk, big rewards?

In the last three years, I have had some good days and many bad days. I can't say that it has been easy. In fact, it has been very difficult.

It is not so important to me to recall all the details of the journey. For this New Year's wish, I choose to focus only on the fact that I educated myself and took care of myself enough to survive that past 1095.75 days. My only job now is to keep going, every day that comes my way, in hopes of spending many more days with my daughter and husband plus my lovely friends.

Happy New Year to all y'all.

Day 1: Cushing's Awareness Month Bloggers Challenge

Cushing's Awareness Month:

Bloggers Challenge

Day 1

Here we are again on April 1st. I join my fellow Cushies in blogging for the 30 days of April in an effort to bring awareness to our disease, Cushing's.

On March 28, 2007, I first saw the word Cushing's while researching thyroid doctors in San Antonio, Texas. Within minutes, I knew the word applied to me. I diagnosed myself with a rare disease on the Internet. I could hardly believe it, and none of the doctors believed it.

These past eight years have been filled with some of the darkest moments of my life. At the time, I wondered if I could make it through the next five minutes. Somehow, I endured the medical challenges before me. I faced disappointment and delay. I have been dismissed and dismayed. I have been tenacious and stubborn in fighting for myself, for what is right.

I admit to being stubborn, refusing to give up the fight when I know the fight is important.

Recently, I have learned to make peace with my disease. No matter what anyone says, you don't get ill and make peace immediately. Peace and uncertainty don't mix. You can't face devastation and immediately say, "Oh well. I guess this is my new normal, and it stinks. There is nothing I can do about it." No no no. That is settling for less than you deserve.

Peace comes with accepting the dire consequences of your disease, facing and befriending death as well as life, and still choosing to walk that line every day with a full heart and the stubbornness that won't let you give up or let Cushing's win.

I strive for peace.

I am 15 months post op BLA, or bilateral adrenalectomy for those with a lot of time on their hands for extra words. 

Another year has gone by. Cortisol is as much a part of my life as ever. Instead of cursing pituitary tumors for high cortisol, I scramble to take my cortisol medicine replacements three times a day. The irony is not lost on me. 

In the coming days, I will tell you more about my life after BLA, and how I navigate this stressful world with no adrenal glands. It's not easy (hint: I take a lot of naps).

I hope that you will stay tuned and learn a little more about the nuances of these cortisol-based diseases of Cushing's (too much) and Addison's (zero). In order to survive, I must understand both.

Sincerely,

Melissa

Cushing's warrior and advocate. 

Pituitary surgery for Cushing's in 06/2009 unsuccessful. 

Pit surgery for Cushing's in 04/2011 unsuccessful. 

Took ketoconazole and suffered through adrenal insufficiency for 14 months. Drug banned in European Union. I stopped postponing my life and chose to get rid of high cortisol.

BLA 12/31/2013 successful. 

I'm fighting to get my life back, and I will win.

Bilateral adrenalectomy for Cushing's disease

I would love to see the whole article because the abstract seems pretty basic.  However, the news is good. Bilateral adrenalectomy or BLA is a good option for patients. Doctors should present this surgery as an option to all patients. It should be our choice to make.

http://www.ncbi.nlm.nih.gov/m/pubmed/25566751/

Bilateral adrenalectomy for Cushing's disease.

Author
Katznelson L¹.

• ¹Departments of Medicine and Neurosurgery, Stanford University School of Medicine, 875 Blake Wilbur Dr MC 5826, Stanford, CA, 94305, USA, lkatznelson@stanford.edu.

Journal

Pituitary. 2015 Jan 8. [Epub ahead of print]

Abstract

PURPOSE: Review the indications, outcomes, and consequences of bilateral adrenalectomy (BLA) in patients with Cushing's disease.

METHODS: A literature review was performed.

RESULTS: The primary therapy for Cushing's disease is surgery, with medical therapy and radiation therapy relegated to an adjuvant role. BLA is indicated in cases of persistent disease following pituitary surgery or in situations where rapid normalization of hypercortisolism is required. When performed via the laparoscopic approach, BLA is associated with a significantly reduced morbidity compared to the traditional, open approach. Following BLA, patients are at risk for adrenal crisis and the concern of Nelson's syndrome. However, BLA leads to a rapid resolution of the signs and symptoms of CS and leads to an improved long-term quality of life.

CONCLUSION: BLA should be considered in the treatment algorithm for patients with persistent CD after failed pituitary surgery, especially in patients who have severe consequences of hypercortisolism or desire pregnancy.

Who ends up going through adrenal crises? Can emotional stress trigger a crisis? Doctors insist it is not possible and advise us to rarely adjust our steroid replacement upwards in times of stress, especially emotional.

I'm please to see this recent article from December 2014 that addresses both questions. And yes, emotional stress can lead to adrenal crisis!!  Just like we Cushies, who adjust our medication daily to keep up with our lives, said. 

http://www.healio.com/endocrinology/adrenal/news/online/%7B779ddc1d-cd37-43cd-9b9e-9d7d7949b8c0%7D/high-rates-of-adrenal-crisis-seen-in-patients-with-chronic-insufficiency

High rates of adrenal crisis seen in patients with chronic insufficiency

Hahner S. J Clin Endocrinol Metab. 2014;doi:10.1210/jc.2014-3191.

• December 8, 2014

Adrenal crisis occurs in a large proportion of patients with chronic adrenal insufficiency, even if they are educated about managing the disease, according to research published in TheJournal of Clinical Endocrinology & Metabolism.

Deaths associated with adrenal crisis occurred in approximately 6% of cases, emphasizing the need for improved management of the life-threatening complication in patients with chronic adrenal insufficiency, German researchers wrote.

"Using for the first time a prospective approach, our study provides unequivocal evidence of a high incidence of [adrenal crisis] in patients with chronic adrenal insufficiency receiving standard replacement therapy," the researchers wrote.

Stefanie Hahner, MD, of the University of Wuerzburg, and colleagues recruited 423 patients with adrenal insufficiency (primary adrenal insufficiency, n=221; secondary adrenal insufficiency, n=202) and followed them for 2 years.

At baseline and every 6 months thereafter, patients were assessed through general questionnaire. Patients received detailed written instructions on glucocorticoid dose adaptation during stress at the outset of the study; those reporting adrenal crisis were contacted by phone.

Entire data sets were available for 364 patients (86%); 64 cases of adrenal crisis during 767.5 patient-years were documented (8.3 crises/100 patient-years).

Gastrointestinal infection, fever and emotional stress accounted for most precipitating causes (20%, respectively); other stressful events — major pain, surgery, strenuous physical activity, heat and pregnancy — or unexplained sudden onset of adrenal crisis also were documented (7%).

Patients with previous adrenal crisis were at higher risk (OR=2.85; 95% CI, 1.5-5.5); no further risk factors were identified.

During follow-up, 10 patients died, and four of these deaths were associated with adrenal crisis (0.5 adrenal crisis-related deaths/100 patient-years).

"Risk factor analysis has limited potential to identify patients at risk for [adrenal crisis], and education in glucocorticoid adjustments for stressful events will not obviate the occasional need for parenteral hydrocortisone to treat impending [adrenal crisis]," the researchers summarized. "Advances in comprehensive patient education including self-administration of parenteral hydrocortisone will be crucial to eliminate death from [adrenal crisis]."

Disclosure: This work was supported by the Else Kröner Fresenius Foundation project grant and the Federal Ministry of Education and Research.

Straight Talk Cushing's

It's good to have choices.

Now there are two FDA-approved drugs available for those Cushies who are not a candidate for a/another surgery but still remain uncured -- Corcept's Korlym and Novartis' Signifor. Two years ago, there were none.

The long-term benefits are clear.

The entire Cushing's community benefits from Big Pharma dollars spent to create what we all want -- disease awareness, earlier diagnosis for all patients, and better treatment options.

To this end, I want to share the fist video that I have seen from Novartis to further this shared mission. It is well done and quite informative.

Share the link and save a life.

http://m.youtube.com/watch?v=Z2vS7sVvx0o&feature=youtu.be

(I tried to embed this video but this action is forbidden by the Novartis youtube channel. Click through. It is an informative video.)

Describe Life after BLA

Nah. I don't feel like it.  I don't want to write down all my happenings. I want to clearly explain everything but that takes time.  I don't feel like explaining things today.

It has been 28 days since my BLA on 12/31/2014.  The day of my surgery, I was given 300 mg of hydrocortisone intravenously.  I was given 150 mg the next day.  I have been tapering down since then. I left the hospital on a dose of 45 mg hydrocortisone at 7:30 am and 15 mg hydrocortisone at 3:00 pm. I added 0.05 mg fludrocortisone, a replacement for the hormone aldosterone, twice a day since I was post-op day 6.  Every week or so, I decrease my hydrocortisone dose to something near 15/5.  I am getting pretty close so that is good. My doctor will like that, because if I take more than my body needs, the extra hydrocortisone gives me Cushing's again. EEK!  NO!

Last Sunday, I dropped my dose again from 20/5 to 17.5/5. I took the former dose for 7 or 8 days. Even that small of a drop takes the wind out of my sails.  On Sunday, I woke at 7:30 am and was back asleep for a nap by 11:00 am.  This, too, will pass.

I discovered that my dear friend Karen shared her post op BLA experience online and she provides clear explanation.

So today, I'll let Karen explain everything. I hope you feel well again soon.

http://m.caringbridge.org/visit/karenthames1/journal/entry/id/594801436/page/1/num/1

Good news 7 days post op BLA

I wanted a quantitative way to track my progress post BLA. My mother, a fantastic seamstress, took my measurements in 12 different places on my body the night before my surgery.  I will use this to monitor the impact of bilateral adrenalectomy and even assess whether I made the right decision for this irreversible treatment. 

I also check in mentally and emotionally each day. I dug out and will restart my gratitude journal that Cushing's emotional imbalance kept me from filling.  I feel happy and content. I'm not filled with rage and frustration, classic symptoms of high cortisol. I am grateful and filled with love. My mind is clear, and my heart is open.  My body and my mind are no longer fighting. They are in harmony. I feel happy.

Cushing's never let me feel that way. 

My body is no longer hanging on to fat because my cortisol is no longer high. Cortisol is the hormone that controls stress response.  High cortisol tells your body to pack on fat to protect vital organs in event of bear mauling or being chased by a lion. 

My abnormal adrenal glands (weighing 10 and 13 grams when normal is 4-6) were spewing cortisol out and flooding my body. My basement and whole house was under water, but even some doctors said no and blocked surgery.  

On New Years Eve, I shut the valve of overflowing cortisol when I was brave enough to trust my body and agree to remove my adrenals. By taking steroid cortisol medication each day, I now take less than my body was making due to the tumors. I take 35 mg hydrocortisone at 7:30 am and 7.5 mg hydrocortisone at 3 pm to replace cortisol made in adrenals. I take 0.05 mg Florinef twice a day to replace the aldosterone hormone also made in the adrenals. I will take these pills, in varying doses, every day for the rest of my life. 

Bilateral adrenalectomy is not the latest weight loss surgery. I am seeing results because tumors took over cortisol production and my body was flooded. Now my body is reacting--as it is built to do--by dropping weight it no longer needs. 

Thank you for your love and support. It's an incredible ride.

-- Melissa 

***Total inches lost***

7 days post op BLA

* measured by seamstress mama
** double checked many times for accuracy

New Chapter of my Life

Hey everyone! I've missed you all. It's not that I haven't. I just haven't even able to keep up on posting like I wish I could. I hope you will always understand why and forgive me.

Today, I'm back with a big update.

I now walk the final  steps towards BLA and a cure from Cushing's. 

After two non-curative transsphenoidal pituitary surgeries, I spent many months wondering what to do and just trying to manage my symptoms. That has become increasing difficult and almost impossible for me as the days and months wore on. 

I started ketoconazole in August 2012 with mixed results. I slept well sometimes but still would wake up at 4:00 am, a Cushie's witching hour.  I felt good at the beginning but it started to wear off.  I spent some hours in the ER on 10/10/12 as I faced severe symptoms of adrenal insufficiency. I needed IV saline and IV hydrocortisone when the injection of 100 mg of Solu-cortef my husband gave me at home wasn't working.  I lost 23 pounds but I felt weak, unfocused, irritable, and overwhelmed. My daytime drowsiness was worse than ever.  I complained to Dr F in June 2013 that I had narcolepsy; I would sit on the couch for only a few moments in the late morning or afternoon, and I would fall asleep instantly. This is after having sleeping six to eight hours. I would just pass out.

In the past few months, I've taken Ritalin at Dr F's suggestion, and it has worked to keep me awake during the day.  I take a long-acting dose in the am and a short acting dose in the early pm. 

I dropped the ketoconazole and a week later, I saw Dr F for an office visit last Monday. He said he needed recent tests before he could clear me for surgery. I tested every day and night until the early hours of Sunday morning.

Midnight cortisol serums

normal is less than 5

diagnostic is greater than 7.5

My results six nights in a row:

5.2, 16.1, 15.4, 8.8, 19.4, 10.8

I am pretty astonished. I had no idea I was high, much less this high. My high test value before was 12.8.   My cortisol has been so high at night that my body is producing zero cortisol during the day. In the past, before testing, my body has been getting only the 20 mg of morning Cortef that I took while I was on Keto and it wasn't enough. My body was unhappy probably because it had become accustomed to such high levels of cortisol. Any drop have me symptoms of adrenal insufficiency: nausea, diarrhea, loss of appetite, extreme fatigue, muscle pain, joint point, grouchiness. 

Now, I am pretty happy to have these test feathers in my rediagnosis cap.  I am waiting for results on four UFCs w/ 17OHS and five salivas taken during the same time period as the high midnight cortisol serums. I feel good that some will be high.  

I hope to be cleared for BLA within the week. There is no visible tumor on my pituitary. Even an exploratory third surgery is not wise, since my second neurosurgeon said surgery would "result in a guaranteed cerebrospinal fluid leak" based on the post operative tissue's location at the bottom of the gland. My doctor and I know that I am not a good fit for other medical therapies. It's BLA time.  I plan on having a BLA before the end of the year, since insurance pays 100% now.

I have done everything I can in the last six years as a Cushing's patient. I have done everything the medical community has required of me, and many things that should not be required:  guessing when to test, knowing what my body was feeling at every moment and what medication would best treat it, watching over every lab technician like a hawk, feeling guilty after outbursts of emotion towards my family and every time I missed family time so I could nap, even keeping up on refills of 15 medications and organizing a 31-day pill case, even when I wasn't sure I was feeling better from any of it. I have suffered in countless MRI machines that could barely hold my large frame but I made it through the hour with some anti-anxiety pills, pain meds, and a one moment at a time attitude.  

This is my road. This is my path. I accept it.  I feel relief and confidence. I am feeling peaceful and hopeful. 

Stay tuned as a march my way towards my cure from the cortisol beast. Thanks for sticking with me.

- Melissa 

Treating Nelson's Syndrome after BLA

Sustained improvements in plasma ACTH and clinical status in a patient with Nelson's syndrome treated with pasireotide LAR, a multireceptor somatostatin analog

Laurence Katznelson, MD
Stanford School of Medicine, Departments of Neurosurgery and Medicine, 875 Blake Wilbur Dr. MC 5821, Stanford, CA 94305-5821

Address all correspondence and requests for reprints to: Laurence Katznelson, MD, Stanford School of Medicine, Departments of Neurosurgery and Medicine, 875 Blake Wilbur Dr. MC 5821, Stanford, CA 94305-5821, lkatznelson@stanford.edu, tel: (650) 721-1020, fax: (650) 736-8100.

• Published online before print March 28, 2013, doi:10.1210/jc.2013-1497 The Journal of Clinical Endocrinology & Metabolism

http://m.jcem.endojournals.org/content/early/2013/03/28/jc.2013-1497.abstract

Abstract

Context: Nelson's syndrome refers to aggressive pituitary corticotroph adenoma growth after bilateral adrenalectomy (BLA) for treatment of Cushing's disease (CD). Pasireotide, a novel somatostatin analog, has been effective in treating CD. Here, the first case report of a patient with Nelson's syndrome treated with pasireotide is presented.

Case Presentation: A 55 yo female was diagnosed with CD in 1973 at age 15 y and underwent BLA 1 year later. She subsequently developed Nelson's syndrome and underwent multiple surgeries, and radiotherapy for adenoma growth.

Following presentation with ocular pain, third cranial nerve palsy, and finding of suprasellar tumor enlargement with hemorrhage, she began pasireotide LAR 60 mg/28 days IM. At baseline, fasting plasma ACTH was 42,710 pg/mL (normal, 5–27) and fasting plasma glucose was 98 mg/dL. After 1 month, ACTH declined to 4,272 pg/mL and has remained stable over 19 months of follow up. Hyperpigmentation progressively improved. MRI scans show reduction in the suprasellar component. Fasting plasma glucose increased to 124 mg/dL and she underwent diabetes management.

Evidence Acquisition and Synthesis:In this clinical case seminar, the current understanding of the treatment of Nelson's syndrome, including use of pasireotide in Cushing's disease, are summarized.

Conclusion: A case of Nelson's syndrome with clinically significant and dramatic biochemical and clinical responses to pasireotide administration is reported. Hyperglycemia was noted following pasireotide administration. Pasireotide may represent a useful tool in the medical management of Nelson's syndrome. Further study of the potential benefits and risks of pasireotide in this population is necessary.

Received February 22, 2013.
Accepted March 25, 2013.
Copyright © 2013 by The Endocrine Society

Cushies can die after exhausting all treatment options

This makes me incredibly sad.  I have had two unsuccessful pituitary surgeries, and I am currently doing medication therapy (pm ketoconazole with am Cortef) until the tumor culprit comes out of hiding and presents itself on the pituitary MRI for a third pituitary surgery.  No one knows how long that will take.

After my first pituitary surgery, I developed ptosis, or drooping eyelid.

**************

Cushing’s: the worst case scenario

Viv Thornton-Jones

Churchill Hospital, Oxford, UK.
Endocrine Abstracts (2008) 15 S58

---

We present the case of a 40-year-old female who was referred to our Department in 1993, for further management following the diagnosis of Cushing’s disease. She proceeded to a transsphenoidal adenenomatectomy (TSA, note: pituitary surgery) which resulted in a biochemical cure.

In 1998 she presented with recurrence of Cushing’s Disease, which was managed by a 2nd TSA (pituitary surgery) followed by external beam irradiation.

Bilateral adrenalectomy followed a year later, due to the inability to control her disease.

In 2001 she presented with Nelson’s Syndrome managed by a 3rd TSA (pituitary surgery) followed this time with Gamma Knife surgery.

In 2004 she presented with manifestations consistent with recurrence of Nelson’s Syndrome and proceeded to a 4th TSA (pituitary surgery). 

Despite the risk of blindness, the patient agreed to a second course of Gamma Knife treatment for the possibility of tumour control.

Over the next 2 years her clinical picture deteriorated, resulting in a right partial ptosis and a sixth nerve palsy.

She was referred to an Oncologist who offered her Chemotherapy, but she refused treatment.

The patient was then in the care of the Palliative Care Team and she died peacefully at home in 2006.

---

Endocrine Abstracts (2008) 15 S58

Thank you to Ami and MaryO for finding this abstract.

A Cushie's Job: Trudging through the Medical Literature

If you have spent any amount of time on this blog, you will know that I am patient advocate who encourages you to be the most informed and self-aware patient you can be.  For us Cushies, especially us cyclical Cushing's patients, it is imperative to be up-to-date on the medical literature.

You also know how difficult that task can be as we fight to keep our energy up for basic daily tasks and chores.  You also remember how Cushing's causes cognitive impairments that just don't give us the right state of mind -- clarity or positivity -- to take on a project this big.

So, I decided to just post stuff here, as I find it.  It may be a repeat (sorry, I probably forgot), or it may be new. Regardless, it will be stuff I am stumbling through as I relearn everything I have to know about Cushing's in order to make my decision.

I am reading through the medical literature for articles like Long-term remission rates after pituitary surgery for Cushing’s disease: the need for long-term surveillance posted by our dear Cushie friend Robin at Survive The Journey.  I am also trying to wade a list of articles my friend Susan recommended about cyclical Cushing's posted on PubMed, the government's database.  These free articles include research done with your tax dollars at the National Institute of Health (NIH). The NIH is considered an authority on Cushing's and even cyclical Cushing's. Based on what I have seen in my five years, the NIH seem to specialize in pediatric Cushing's cases more than adults, i.e. I see more pediatric patients than adult patients accepted for testing and treatment at the NIH.

So, I have to make my way through the medical literature about:

• quality of life for patients after several pituitary surgeries vs. quality of life for those who chose BLA
• remission rates for each type of treatment (3rd pituitary surgery vs BLA)
• enzymes and other hormones produced in the pituitary that may vanish after repeated pituitary surgery
• patient success stories for each

I mean, all this and a bag of chips. EVERYTHING!  I read a lot of this stuff in the beginning. It was "this could happen to me" reading, so the shock resonated longer than the information. Meaning, the scariness stuck when the facts didn't. 

Now, I find my mind so hazy that I just can't read through these medically-rigorous articles anymore. I really struggle.
I can't remember what I read already.
I can't find something I already found before.
I can't even think of good keywords to search.
I've already made a million laps around the internet.

It's a mess. I AM A MESS!

So, being that it is my blog, this is what I'm gonna do.  I plan to "store" articles here that I am reviewing for my own benefit. If you see something that you have never seen before, well, you benefit, too.

HEY!  THEN MAYBE YOU CAN MAKE MY DECISION FOR ME!

Chanelle hits the news again

I just love this fireball Chanelle.  You have seen her featured here in several posts (here, here, here, and here). Well, here she is again, and I'm very proud of her. This brought tears to my eyes. Keep going, Chanelle!  ~mm

Grad perseveres to earn degree

May 8, 2012 12:10 am

Chanelle Felder's struggle with Cushing's syndrome prompted her to pursue a career as a patient advocate. Felder took ballet in 2008, during her fight with disease. Chanelle Felder battled cyclical Cushing's syndrome in high school. She graduates from Germanna Community College this week and is preparing to pursue a bachelor's degree at a Virginia university this fall.

By PAMELA GOULD

Chanelle Felder found her mission in life through the mysterious illness that left her mind in a fog, her body bloated and her high-energy lifestyle on hold.

"I was directionless before," the 22-year-old said. "Now I have a laserlike focus on what I want my legacy to be and what I want to get out of life."

Felder, who graduates from Germanna Community College on Wednesday, was 16 when her slender 5-foot, 7-inch frame started expanding inexplicably.

She began having crying spells, waking in the middle of the night, and suffering numbness and pain in the feet that for years had carried her gracefully across dance floors.

Clumps of hair started falling out, she became sluggish, and her normally sharp mind started going blank.

Felder was a Mountain View High School junior, cheerleader and honors student when the symptoms began. She found them shocking and frightening.

Doctors offered possible diagnoses such as a thyroid disorder, but none fit until the North Stafford teen stumbled upon a program on the Discovery Health channel.

"My turning point was an episode of 'Mystery Diagnosis,'" she said.
A woman named Sharmyn McGraw was describing the symptoms of Cushing's disease. Felder immediately saw her own situation.

She went online, did research and told her parents that's what she had. The rare disorder afflicts 10 to 15 of every 1 million people. It's even rarer in children and adolescents.

Though her parents supported her self-diagnosis, doctors were slower to get on board. But after medical tests confirmed it, she underwent brain surgery in September 2007.

That first surgery removed a benign tumor from her pituitary gland. However, the symptoms returned, prompting a second brain surgery to remove more tumors in January 2009.

It turned out that she had cyclical Cushing's syndrome, a condition in which the symptoms disappear and then return.

When the second surgery didn't resolve the problems, Felder opted to have both adrenal glands removed in April 2010.

That procedure eliminated the Cushing's symptoms because it's driven by the hormones produced by those glands, which sit atop the kidneys.

But removal of the adrenals meant she was without the hormones they produce, which, among other things, help people cope with stress.

She now takes four medications daily to regulate her endocrine system and keeps a close watch on her stress level. But her bubbly personality and energy are back.

MOVING FORWARD

Felder graduated from Mountain View High in June 2008, nine months after her first surgery.
Then, on a doctor's advice, she waited a year to start college.

Her parents urged her to ease into her courses, so it's taken her three years. However on Wednesday, Felder will walk across the stage at the Fredericksburg Expo and Conference Center to receive her associate degree in arts and sciences.

"She's been inspirational to other students," said Judi Johnson-Bartlett, coordinator and student adviser at Germanna's Stafford Center.

Johnson-Bartlett rattled off adjectives to describe Felder: determined, hard-working, dedicated, persistent. "The sky is the limit for her."

Felder majored in science while taking classes on the Germanna campus in Spotsylvania County and at the recently opened Stafford Center.

She frequently drew on her experience for class projects and presentations.

The time spent researching her diagnosis and staying abreast of the steps in her treatment gave her an understanding of medical science and terminology she wouldn't have learned otherwise.

It also redirected her interest from a career in social work to one in which she plans to serve as a patient advocate.

If it hadn't been for a dash of teenage defiance, the support of her parents, and the prayer and encouragement of people at Mount Ararat Baptist Church, Felder said, she'd probably still be suffering.

That's why she wants to study the dietetic field next fall when she attends Virginia Tech or James Madison University and why she's also interested in communications.

"My ultimate legacy will be to get information out about this disease and about other diseases like this," Felder said.

She also wants to provide the support for others that she received during her medical ordeal.

She's already part of an online network of Cushing's patients who share their stories; some of them have been heartbreaking.

She said some people have been ostracized by relatives who don't understand their symptoms, or worse yet, suggest they're lying and just lazy when their weight balloons and they lack energy.

Others have died from symptoms related to the disorder.

Many, she said, just resign themselves to a life of suffering after medical professionals brush off their symptoms or aren't familiar with the disorder.

Felder said her mission crystallized as a result of Facebook communications with a man in California with Cushing's syndrome.

She shared the treatment she'd undergone, and in her he found hope and committed to the same path.

He underwent surgery and messaged her recently to say, "You saved my life."

Felder became teary as she shared that encounter.

"Just to think I was just a 16-year-old girl. For me to go through that and actually help someone, it made me see it wasn't in vain," she said, pausing to keep her composure.

"That's why I went through it--to help people."

---

Germanna Community College will hold its spring graduation ceremony at the Fredericksburg Expo and Conference Center on Wednesday at 7 p.m.

Chanelle Felder is scheduled to sing the national anthem as part of the commencement exercises.

---

Copyright 2012 The Free Lance-Star Publishing Company.


A special thanks to Pamela Gould (540/735-1972, pgould@freelancestar.com) for the excellent story.

Day 3: Around the Cushie World in 30 Days~10 Things I Can't Live Without

For today's featured post, I share with you Robin's post entitled 10 Things I Can't Live Without.  

I find this post particularly important for Cushies, not in the nice-to-know way but the you-can-die-without-it way.  The pituitary gland and adrenal glands' choreographed dance to make cortisol is life-sustaining.  Enter a tumor and friends to tinker with that, it the dance turns ugly real quick.

You see, when people in the general populace talk about things they can't live without, they aren't talking real life and death. They are talking, I'd throw a bitch fit without it, it would make me cranky, I'd be bummed out if... 

Big difference.

We love Robin. She is a science teacher who helps all of us so much.  You can read her blogs at Survive the Journey and 365 with Cushing's.

Day 20: Surgiversary

DATE:  
APRIL 20, 2011.


TARGET:  
3 MILLIMETER TUMOR ON THE RIGHT SIDE OF PITUITARY.


OFFENSE:  

UNLAWFUL PRODUCTION OF EXCESS ACTH, SPURRED EXCESS PRODUCTION OF CORTISOL BY ADRENAL GLANDS.


ACCOMPLICE:  

ADRENAL GLANDS FOR AIDING AND ABETTING.


STRATEGY:  

TRANSSPHENOIDAL TRANSNASAL RESECTION OF PITUITARY TUMOR.


OUTCOME:  

1) SUCCESS. PATIENT ALIVE. TUMOR REMOVED.


2) FAIL. PITUITARY HORMONE ACTH STILL HIGH ON POST OP DAYS 5 & 16. TUMOR CELLS PLAYING LETHAL GAME OF PEEK-A-BOO. 


3) FAIL. NOT ONE DAY OF RELIEF FROM CUSHING'S SYMPTOMS. PATIENT CONTINUES STRUGGLES TO FIGHT DAMAGE TO BODY, MIND & SOUL CAUSED BY EXCESS ACTH & CORTISOL PRODUCTION.

4) PATIENT STRUGGLES TO RE-ENGAGE IN LIFE AND SOCIETY WITH MULTIPLE HORMONE- AND VITAMIN-DEFICIENCIES (GROWTH HORMONE DEFICIENCY, VITAMIN D DEFICIENCY, OSTEOPENIA, THYROID DEFICIENCY, FERRITIN (IRON) DEFICIENCY, ACTH EXCESS, CORTISOL EXCESS). 

5) PATIENT DECIDES ON NEXT COURSE OF TREATMENT: 

3RD PITUITARY SURGERY vs. BILATERAL ADRENALECTOMY vs. KORLYM

Structures surrounding pituitary. Important things nearby include carotid artery and optic nerve.

Arrow points to 3 millimeter tumor, right side pituitary; black indented area on opposite side shows space where tumor removed in 1st surgery (6/2009).

Recovery. Swelling but not too bad. Rocking those eye brows and ACTH shadow (acanthosis nigricans). 

Post op day 4 with Lola's blanket. Neurosurgeon deflated,

removed balloons from nose. Balloons measured 4-6 inches.

Crazy nurses insisted on placing an IV here. Btw--my veins are good.

CUSHING’S: Relentless and Nefarious

I am 27 days post op for my second pituitary surgery. I had blood work done on post op day 16 after withholding for 24 hours, aka skipping my afternoon dose of hydrocortisone the day before.  Late last Friday, I received some terrible news from my lab results.  My body is making too much cortisol and ACTH so soon after pituitary surgery.

04/24/2011 post op day 5 @ 8 am (withheld pm dose on previous day)
cortisol 13.6 (4-22)
acth 24 (5-27)
glucose 96 (65-99)
sodium 142 (135-146)

05/06/2011 post op day 16 @ 8 am (withheld pm dose on previous day)
cortisol 17.1 (4-22)
acth 59 (5-27)
glucose 102 (65-99)
sodium 142 (135-146)

       My cortisol increased to 17.1 in the am, and ACTH is more than twice normal. I did capture some high ACTH values prior to my first pituitary surgery, with one high ACTH during the day, and that was 125 baseline for IPSS. Some daytime values were in the high 20s but not like this. My 4 am ACTH in 2007 were 78 and 105.

 ** ** ** ** ** ** ** **

       My friend Debra reminded me that a cure for Cyclical Cushing’s is very uncertain, often as low as 30% for the first pituitary surgery. She reviewed the medical literature and read every single study she could find on remission after pituitary surgery for Cushing’s. The highest cortisol value she could find that indicated remission was 14 (post op day 5 cortisol level while withholding HC dose for 24 hours). Mine are 13.6 and 17.1.  Debra pointed to this European Journal of Endocrinology article entitled The Prevalence and Characteristic Features of Cyclicity and Variability in Cushing's Disease, which was published in 2009.
       The second pituitary surgery did not cure my Cushing's.  There is no other conclusion. I am just heartbroken and devastated about this medical development, even though I have known it was a possibility since 2007.
Cushing’s patients whose bodies will not stop producing cortisol must remove both adrenal glands in order to ultimately stop all cortisol production at the source. Since high cortisol breaks down many body systems, it just not an option to let Cushing’s keep going.  So, we trade Cushing’s and high cortisol for Addison’s and low cortisol. REPEAT: I will trade the terribly relentless and rollercoaster cortisol levels of Cyclical Cushing's disease for the unpredictable make-no-cortisol-at-all and hope-there-is-no-emergency-or-trauma Addison's.  I am now making plans to have both adrenal glands removed in a procedure called a bilateral adrenalectomy (BLA). NYU’s Department of Surgery posted information on the adrenalectomy procedure.
       Last month, I posted a list of Cushie Warriors – 50 people from around the world who have had to have multiple surgeries in an attempt to rid themselves of Cushing’s. I wish we could get a restraining order for this Cushing’s menace.
       My current dose of hydrocortisone is 15 mg at 8 am and 5 mg at 1 pm.  I will taper that dose responsibly but quickly after speaking to my Cushing’s endocrinologist this Wednesday. 
       That’s all I have to say about that.
        
        Melissa

Adrenal Crisis Letter

When multiple pituitary surgeries fail to resolve the symptoms, Cushing's patients often have to move forward by having an operation to remove their adrenal glands--the maker of cortisol. They must carry around an adrenal crisis letter explaining their condition with them at all times. This will notify medical professionals about the care that is required for us. Often, a person in adrenal crisis can not think clearly or advocate for themselves. Adrenal insufficiency or adrenal crisis is very serious. It requires a trip to the emergency room for immediate care.

Thanks to you, Robin, for sharing this information on your 365 Days with Cushing's blog. We wish you the best for your upcoming bilateral adrenalectomy (BLA).

~Melissa

HOW WOULD IT FEEL TO HAVE ADDISON'S: Wondering about life after a BLA

I have mentioned that I am facing a Cushing's reoccurrence. I have to make the difficult decision: try a second pituitary surgery or go straight to BLA, or bilateral adrenalectomy.

Fellow Cushie Gina posted Living with Addison's Disease: An Owner's Manual for Individuals with this Disease, and I wanted to share it. You may also download the owner's manual in pdf format, too.

I haven't read this yet, but I've downloaded it on my iPhone and plan to read it all! I will return and post about how this compares to the patients' experiences we read about on the Cushing's Help and Support message boards. Knowing what I know about Gina, I bet it will be a good read.

~melissa