🦓 Day 15, Cushing’s Awareness Challenge 2022

Today’s Cushing’s Awareness Challenge post is about kidney cancer (renal cell carcinoma). You might wonder how in the world this is related to Cushing’s. I think it is, either directly or indirectly.

I alluded to this a couple days ago when I said:

I finally started the Growth Hormone December 7, 2004.
Was the hassle and 3 year wait worth it?
Stay tuned for tomorrow, April 15, 2016 when all will be revealed.

So, as I said, I started Growth Hormone for my panhypopituitarism on December 7, 2004.  I took it for a while but never really felt any better, no more energy, no weight loss.  Sigh.

April 14 2006 I went back to the endo and found out that the arginine test that was done in 2004 was done incorrectly. The directions were written unclearly and the test run incorrectly, not just for me but for everyone who had this test done there for a couple years. My endo discovered this when he was writing up a research paper and went to the lab to check on something.

So, I went off GH again for 2 weeks, then was retested. The “good news” was that the arginine test is only 90 minutes now instead of 3 hours.

Wow, what a nightmare my arginine retest started! I went back for that Thursday, April 27, 2006. Although the test was shorter, I got back to my hotel and just slept and slept. I was so glad that I hadn’t decided to go right home after the test.

Friday I felt fine and drove back home, no problem. I picked up my husband for a biopsy he was having and took him to an outpatient surgical center. While I was there waiting for the biopsy to be completed, I started noticing blood in my urine and major abdominal cramps.

There were signs all over that no cellphones were allowed so I sat in the restroom (I had to be in there a lot, anyway!) and I left messages for several of my doctors on what I should do. It was Friday afternoon and most of them were gone 🙁  I finally decided to see my PCP after I got my husband home.

When Tom was done with his testing, his doctor took one look at me and asked if I wanted an ambulance. I said no, that I thought I could make it to the emergency room ok – Tom couldn’t drive because of the anaesthetic they had given him. I barely made it to the ER and left the car with Tom to park. Tom’s doctor followed us to the ER and instantly became my new doctor.

They took me in pretty fast since I was in so much pain, and had the blood in my urine. At first, they thought it was a kidney stone. After a CT scan, my new doctor said that, yes, I had a kidney stone but it wasn’t the worst of my problems, that I had kidney cancer. Wow, what a surprise that was! I was admitted to that hospital, had more CT scans, MRIs, bone scans, they looked everywhere.

My new “instant doctor” felt that he wasn’t up to the challenge of my surgery, so he called in someone else.  My next new “instant doctor” came to see me in the ER in the middle of the night.  He patted my hand, like a loving grandfather might and said “At least you won’t have to do chemotherapy”.  And I felt so reassured.

It wasn’t until later, much after my surgery, that I found out that there was no chemo yet that worked for my cancer.  I was so thankful for the way he told me.  I would have really freaked out if he’d said that nothing they had was strong enough!

My open radical nephrectomy was May 9, 2006 in another hospital from the one where the initial diagnosis was made. My surgeon felt that he needed a specialist from that hospital because he believed preop that my tumor had invaded into the vena cava because of its appearance on the various scans. Luckily, that was not the case.

My entire left kidney and the encapsulated cancer (10 pounds worth!) were removed, along with my left adrenal gland and some lymph nodes. Although the cancer (renal cell carcinoma AKA RCC) was very close to hemorrhaging, the surgeon believed he got it all.

He said I was so lucky. If the surgery had been delayed any longer, the outcome would have been much different. I will be repeating the CT scans every 3 months, just to be sure that there is no cancer hiding anywhere. As it turns out, I can never say I’m cured, just NED (no evidence of disease). This thing can recur at any time, anywhere in my body.

I credit the arginine re-test with somehow aggravating my kidneys and revealing this cancer. Before the test, I had no clue that there was any problem. The arginine test showed that my IGF is still low but due to the kidney cancer I couldn’t take my growth hormone for another 5 years – so the test was useless anyway, except to hasten this newest diagnosis.

So… either Growth Hormone helped my cancer grow or testing for it revealed a cancer I might not have learned about until later.

My five years are up now.  When I was 10 years free of this cancer my kidney surgeon *thought* it would be ok to try the growth hormone again.  I was a little leery about this, especially where I didn’t notice that much improvement.

What to do?

BTW, I decided to…

⁉️ Cushing’s Myths and Facts: “All types of Cushing’s are the same”

Myth: “All types of Cushing’s are the same”

myth-busted

Fact: In the words of our dear friend and advocate, Robin Ess, “There are many genetic varieties with quite a few discovered in the past couple of years. Plus, there are several types such as adrenal, ectopic, and pituitary. And so on”….Amazingly, some doctors do not realize that there are different varieties of Cushing’s and that the symptoms can come from a different source.

For instance, a doctor might rule out a pituitary tumor and completely dismiss the patient, even with biochemical evidence of Cushing’s. That doctor, instead of dismissing the patient, should thoroughly look for other potential sources, such as an adrenal tumor, or yet another source. Did you know that tumors on one’s lungs can even cause Cushing’s? Most people don’t know that.

For more information about the different types of Cushing’s, please read: http://www.mayoclinic.org/diseases-conditions/cushing-syndrome/basics/causes/con-20032115

Another great article regarding ectopic Cushing’s can be found here: http://www.nejm.org/doi/full/10.1056/NEJM199809243391304#.VH-80v5f2s8.facebook

MaryONote: Folks might be interested in listening to this podcast episode with Jayne, a Cushing’s patient who had pituitary surgeries and a bilateral adrenalectomy before finding the true source of her ectopic Cushing’s – lung tumors.

http://www.blogtalkradio.com/cushingshelp/2008/01/31/tentative-date-an-interview-with-jayne-cyclical-cushings-patient

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⁉️ Cushing’s Myths and Facts: Cushing’s Syndrome/Disease can be healed or cured

Myth: Cushing’s Syndrome/Disease can be healed or cured through change in diet or exercise.

myth-busted

Fact: NO! Caloric intake or lack of exercise has NO impact on weight gain and/ or loss in persons with Cushing’s.

Saying that someone “cheated” on their diet may seem reasonable to some as a reason for weight gain but I assure you that a candy bar or a piece of pie does not make a person with Cushing’s gain weight or get sick. Excess cortisol is the reason for Cushing’s symptoms. Treating the disease is the only way to alleviate symptoms.

The first line of treatment with the highest rate of remission is currently surgery to remove the tumor (s) from the pituitary, adrenal gland, or ectopic source.

🦓 Day 5, Cushing’s Awareness Challenge 2022

In Day 9 on April 9, 2015, I wrote about how we got the Cushing’s colors of blue and yellow.  This post is going to be about the first Cushing’s ribbons.

I was on vacation  in September, 2001 when SuziQ called me to let me know that we had had our first Cushie casualty (that we knew about).

On the message boards, Lorrie wrote: Our dear friend, Janice died this past Tuesday, September 4, 2001. I received an IM from her best friend Janine, tonight. Janine had been reading the boards, as Janice had told her about this site, and she came upon my name and decided to IM me. I am grateful that she did. She said that she knew that Janice would want all of us to know that she didn’t just stop posting.

For all of the newcomers to the board that did not know Janice, she was a very caring individual. She always had something positive to say. Janice was 36 years old, was married and had no children. She had a miscarriage in December and began to have symptoms of Cushing’s during that pregnancy. After the pregnancy, she continued to have symptoms. When discussing this with her doctor, she was told that her symptoms were just related to her D&C. She did not buy this and continued until she received the accurate diagnosis of Cushing’s Syndrome (adrenal) in March of 2001. Tragically, Janice’s tumor was cancerous, a very rare form of Cushing’s.

Janice then had her tumor and adrenal gland removed by open adrenalectomy, a few months ago. She then began chemotherapy. She was very brave through this even though she experienced severe side effects, including weakness and dizziness. She continued to post on this board at times and even though she was going through so much, she continued with a positive attitude. She even gave me a referral to a doctor a few weeks ago. She was my inspiration. Whenever I thought I had it bad, I thought of what she was dealing with, and I gained more perspective.

Janice was having difficulty with low potassium levels and difficulty breathing. She was admitted to the hospital, a CT scan was done and showed tumor metastasis to the lungs. She then was begun on a more aggressive regimen of chemo. She was discharged and apparently seemed to be doing well.

The potassium then began to drop again, she spiked a temp and she was again admitted to the hospital. She improved and was set to be discharged and then she threw a blood clot into her lungs. She was required to be put on a ventilator. She apparently was at high risk for a heart attack. Her husband did not want her to suffer anymore and did not want her to suffer the pain of a heart attack and so chose for the doctors to discontinue the ventilator on Tuesday. She died shortly thereafter.

Janice was our friend. She was a Cushie sister. I will always remember her. Janine asked me to let her know when we get the Cushing’s ribbons made as she and the rest of Janice’s family would like to wear them in her memory. She said that Janice would want to do anything she could to make others more aware of Cushing’s.

The image at the top of the page shows the first blue and yellow ribbon which were worn at Janice’s funeral.  When we had our “official ribbons” made, we sent several to Janice’s family.

Janice was the first of us to die but there have been more, way too many more, over the years.  I’ll write a bit more about that on Day 21.

Basics: Adrenal Surgery: One Patient’s Experiences

Extracted and adapted from this series: https://cushings.invisionzone.com/topic/51040-on-my-way-to-getting-well/

Post 1) I was officially diagnosed with Cushing’s yesterday. I have a CT scan to check on my adrenal tumor and a meeting with my surgeon tomorrow. Hopefully they will schedule surgery for Monday or Tuesday. I have suffered over a year with this, been in congestive heart failure, and believe this cortisol caused my son to be stillborn in March. It’s been the year from hell. Please pray that all goes well tomorrow and that I will be cured of this once and for all!!

Post 2) Surgery set for the 23rd!!!!! He is planning a right adrenaltectomy. I am so darn excited…

Post 3) I’m almost two weeks out of adrenal surgery. He removed the tumor & my gland. This has been the hardest and most painful two weeks of my life. I am already noticing little changes in my body. My skin is getting texture, my hair is not as brittle, my swelling goes down each day, and my nails are white instead of yellow and are stronger. I am getting hair back on my arms, legs, & feet too. I can’t wait to continue to get well. I am ready to be able to get out and about. I am pretty much housebound now because of the pain of the withdrawal from the cortisol. I stay on my painkillers and rest in my recliner. Hubby bought it for me because I can’t sleep in the bed comfortably. He’s the best. He’s been sleeping on our air mattress in the living room with me for almost 2 weeks now. He is always there to help me get out of the recliner when I need to. He is amazing. Just wanted to update you all. Getting better everyday.

Post 4) I am on 40mg Hydrocortisone daily right now. I will have my first wean close to Christmas. I have an appt. on the 21st with my endo. She is fantastic and saved my life from this stuff. I am so blessed. Today is a rough day. I did have 2 good days in a row which was a huge blessing. Thanks for thinking of me!

Post 5) Well, I just survived month 1 of recovery. It was HORRIBLE. I have never had so much pain in my life. I am still on 40 mg and my endo. wants me to wean 10 mg starting on the 27th. We’ll see how it goes. I have so much pain, shaking, chills, no sleep NOW. I can’t imagine how its going to be on a lower dose. My cortisol level was SO HIGH (2107) before surgery. I knew this withdrawal was going to be terrible. SHe had never seen a level as high as mine before. The lab actually tested my urine twice because they didn’t believe it the first time. I am doing a lot of resting right now. I am very nervous about my mother leaving on New Year’s Day. I don’t know how I am going to handle my 3 year old on my own. I hurt so badly and my vision isn’t the greatest yet. Thanks for thinking of me and writing me back.

Post 6) We have another call into my endo about my suffering. I have done nothing but shake uncontrollably all day so far. I hurt so badly. I am up every hour at night writhing in pain. I refuse to suffer like this anymore. I want some relief. Thank you so much for all of the advice. It means the world to me. Great news is that I am off my BP meds as of today!! Cardiologist’s office said I could quit them. I am thrilled. Now to get this pain under control.

Post 7) Endo said we can do whatever I can tolerate. I am now doing 20/20/10 instead of 20/10/10. I am still in pain, but it’s a little more tolerable. She said if I am just miserable and can’t take the pain, then I can do a bedtime dose. I am going to try melatonin to help me sleep per her suggestion. She wants to see how I do on this new dose and start a slow wean in a few weeks.

Post 8) Things have been getting better by the week. New years day was my best physical and mental day so far. I can actually feel my old self returning! !! Today I have lots of bone/muscle pain. Its better than a few weeks ago by far. Yesterday I was able to enjoy my son and play with him for the first time in a long time. I could even dance a little with him. He was so happy. I am down to 20/17.5/10& am handling it well. The pain is tolerable. My hump is almost gone, my stomach is mushy and shrinking, skin is peeling and improving, hair is growing in normally. I will be six weeks out this Wed.

 

ℹ️ Basics: Cushing’s Syndrome vs Cushing’s Disease

What is Cushing’s syndrome?

Any condition that causes the adrenal gland to produce excessive cortisol results in the disorder Cushing’s syndrome. Cushing syndrome is characterized by facial and torso obesity, high blood pressure, stretch marks on the belly, weakness, osteoporosis, and facial hair growth in females.

Cushing’s syndrome has many possible causes including tumors within the adrenal gland, adrenal gland stimulating hormone (ACTH) produced from cancer such as lung cancer, and ACTH excessively produced from a pituitary tumors within the brain. ACTH is normally produced by the pituitary gland (located in the center of the brain) to stimulate the adrenal glands’ natural production of cortisol, especially in times of stress.

When a pituitary tumor secretes excessive ACTH, the disorder resulting from this specific form of Cushing’s syndrome is referred to as Cushing’s disease.

As an aside, it should be noted that doctors will sometimes describe certain patients with features identical to Cushing’s syndrome as having ‘Cushingoid’ features. Typically, these features are occurring as side effects of cortisone-related medications, such as prednisone and prednisolone.

ℹ️ Cushing’s Basics: The Endocrine System

The endocrine system is a complex network of glands and organs. It uses hormones to control and coordinate your body’s metabolism, energy level, reproduction, growth and development, and response to injury, stress, and mood. The following are integral parts of the endocrine system:

 

  • Hypothalamus. The hypothalamus is located at the base of the brain, near the optic chiasm where the optic nerves behind each eye cross and meet. The hypothalamus secretes hormones that stimulate or suppress the release of hormones in the pituitary gland, in addition to controlling water balance, sleep, temperature, appetite, and blood pressure.
  • Pineal body. The pineal body is located below the corpus callosum, in the middle of the brain. It produces the hormone melatonin, which helps the body know when it’s time to sleep.
  •  Pituitary . The pituitary gland is located below the brain. Usually no larger than a pea, the gland controls many functions of the other endocrine glands.
  • Thyroid and parathyroid. The thyroid gland and parathyroid glands are located in front of the neck, below the larynx (voice box). The thyroid plays an important role in the body’s metabolism. The parathyroid glands play an important role in the regulation of the body’s calcium balance.
  • Thymus. The thymus is located in the upper part of the chest and produces white blood cells that fight infections and destroy abnormal cells.
  •  Adrenal gland . An adrenal gland is located on top of each kidney. Like many glands, the adrenal glands work hand-in-hand with the hypothalamus and pituitary gland. The adrenal glands make and release corticosteroid hormones and epinephrine that maintain blood pressure and regulate metabolism.
  •  Pancreas . The pancreas is located across the back of the abdomen, behind the stomach. The pancreas plays a role in digestion, as well as hormone production. Hormones produced by the pancreas include insulin and glucagon, which regulate levels of blood sugar.
  • Ovary. A woman’s ovaries are located on both sides of the uterus, below the opening of the fallopian tubes (tubes that extend from the uterus to the ovaries). In addition to containing the egg cells necessary for reproduction, the ovaries also produce estrogen and progesterone.
  • Testis. A man’s testes are located in a pouch that hangs suspended outside the male body. The testes produce testosterone and sperm.

❓Clinical Trial: Congenital Adrenal Hyperplasia (CAH)


The CAHmelia clinical trials are exploring a new investigational treatment for classic CAH.

CAHmelia 203 and CAHmelia 204 are clinical trials to test tildacerfont in adults with classic CAH, which may offer you and your loved ones hope of a brighter future – one where you may not have to choose between symptom management and long-term health.
Tildacerfont is a new type of oral, once-daily investigational treatment – one that is not a steroid – that is currently being tested in adults with classic CAH. By reducing the amount of androgens your body makes, tildacerfont may improve your classic CAH symptoms.
This investigational treatment will not replace your steroid treatment but may allow you to manage your disease with lower amounts of steroids at normal or near-normal doses.

Who can take part in this trial? You may be able to take part if you:Are at least 18 years of ageHave a confirmed diagnosis of classic CAH due to 21-OH deficiencyHave been on the same daily dose of steroids (GCs and/or mineralocorticoids) for at least 1 month before starting the trialBoth trials are now open for enrollment.
Tildacerfont is an investigational treatment not authorized for use in people outside the clinical trial.
For more information, go to: clarahealth.com/studies/cahmelia

​​​​​​​ 

ℹ️ Basics: What is Cortisol?

Cortisol is a hormone which produced by the adrenal gland (cortex) to control blood sugar. The production of cortisol is triggered by the pituitary hormone ACTH. Cortisol is a glucocorticoid which stimulates an increase in blood glucose. Cortisol will also stimulate the release of amino acids from muscle tissue and fatty acids from adipose tissue. The amino acids are then converted in the liver to glucose (for use by the brain). The fatty acids can be used by skeletal muscles for energy (rather than glucose) thereby freeing up glucose for selective utilization by the brain. Cortisol levels are often measured to evaluate the function of the pituitary or adrenal glands. Some of the cortisol is metabolized by the liver to produce 17 hydroxycorticosteroids, which is then excreted in the urine.

The primary stress hormone. Cortisol is the major natural GLUCOCORTICOID (GC) in humans.

Synthetic cortisol, also known as hydrocortisone, is used as a drug mainly to fight allergies and inflammation.

Physiology
The amount of cortisol present in the serum undergoes diurnal variation, with the highest levels present in the early morning, and lower levels in the evening, several hours after the onset of sleep. Information about the light/dark cycle is transmitted from the retina to the paired suprachiasmatic nuclei in the hypothalamus. Changed patterns of the serum cortisol levels have been observed in connection with abnormal ACTH levels, clinical depression, psychological stress, and such physiological stressors as hypoglycemia, illness, fever, trauma, surgery, fear, pain, physical exertion or extremes of temperature. There is also significant individual variation, although a given person tends to have consistent rhythms.

Cortisol also inhibits the secretion of corticotropin releasing hormone (CRH), resulting in feedback inhibition of ACTH secretion. Some researchers believe that this normal feedback system may break down when animals are exposed to chronic stress.

In normal release, cortisol has widespread actions which help restore homeostasis after stress. It acts as a physiological antagonist to insulin by promoting gluconeogenesis, breakdown of lipids, and proteins, and mobilization of extrahepatic amino acids and ketone bodies. This leads to increased blood glucose concentrations, resulting in increased glycogen formation in the liver (Freeman, 2002). It also increases blood pressure. In addition, immune and inflammatory cells have their responses to stress attenuated by cortisol, and the hormone thus lowers the activity of the immune system. Bone formation is also lowered by cortisol.

These normal endogenous functions are the basis for the physiological consequences of chronic stress – prolonged cortisol secretion causes muscle wastage, hyperglycemia, and suppresses immune / inflammatory responses. The same consequences arise from long-term use of glucocorticoid drugs.

Also, long-term exposure to cortisol results in damage to cells in the hippocampus. This damage results in impaired learning. However, short-term exposure of cortisol helps to create memories; this is the proposed mechanism for storage of flash bulb memories.

Pharmacology
As an oral or injectable drug, cortisol is also known as hydrocortisone. It is used as an immunosuppressive drug, given by injection in the treatment of severe allergic reactions such as anaphylaxis and angioedema, in place of prednisolone in patients who need steroid treatment but cannot take oral medication, and peri-operatively in patients on long-term steroid treatment to prevent an Addisonian crisis.

It is given by topical application for its anti-inflammatory effect in allergic rashes, eczema and certain other inflammatory conditions. It may also be injected into inflamed joints resulting from diseases such as gout.

Compared to prednisolone, hydrocortisone is about 1/4th the strength. Dexamethasone is about 40 times stronger than hydrocortisone. For side effects, see corticosteroid and prednisolone.

A certain amount of cortisol is necessary for life. Without cortisol even a small amount of stress will kill you. Addison’s disease is a disease which causes low cortisol levels, and which is treated by cortisol replacement therapy.

Cortisol…

  • helps maintain blood pressure and cardiovascular function;
  • helps slow the immune system’s inflammatory response;
  • helps balance the effects of insulin in breaking down sugar for energy; and
  • helps regulate the metabolism of proteins, carbohydrates, and fats.

 

📞Webinar on The 20-minute Mini Back Scope Adrenalectomy (MBSA)

 

Dr. Friedman will host Tobias Carling, MD, PhD, FACS
Surgeon-in-Chief & Founder
Carling Adrenal Center
Hospital for Endocrine Surgery
www.adrenal.com
Who will talk on:
The 20-minute Mini Back Scope Adrenalectomy (MBSA)

The Carling Adrenal Center is the world’s busiest adrenal surgery center, operating on patients from all 50 states and all over the world.

Dr. Carling is the most experienced adrenal surgeon in the United States, and by far the world’s most knowledgeable surgeon-scientist when it comes to adrenal gland function and disease, adrenal tumors and cancer, and all forms of adrenal gland surgery. Dr. Carling has more experience with advanced minimally invasive adrenal and endocrine operations than any surgeon in the United States. A fellow of the American College of Surgeons, Dr. Carling is a significant member of both the American Association of Endocrine Surgeons (AAES) and the International Association of Endocrine Surgeons (IAES).

Dr. Carling spent 17.5 years at Yale University and the Yale University School of Medicine where he served as the Chief of Endocrine Surgery, Associate Professor of Surgery, Program Director of the Yale Endocrine Surgery Fellowship and the Founder & Director of the Yale Endocrine Neoplasia Laboratory, a supreme scientific program focused on the molecular pathogenesis of tumors arising in the adrenal, thyroid and parathyroid glands.

Dr. Carling moved his world-renowned adrenal surgery program to Tampa, Florida in early 2020 to start the Carling Adrenal Center. Here, patients needing adrenal surgery have access to the best practices and best techniques the world has to offer.

Dr. Carling works closely with Dr. Friedman and will be able to perform a Mini Back Scope Adrenalectomy with a referral from Dr. Friedman.
Sunday • November 7• 6 PM PST

Via Zoom Click here to join the meeting or
https://us02web.zoom.us/j/4209687343?pwd=amw4UzJLRDhBRXk1cS9ITU02V1pEQT09
OR
+16699006833,,4209687343#,,,,*111116#
Slides will be available before the webinar and recording after the meeting at slides

Your phone/computer will be muted on entry. There will be plenty of time for questions using the chat button.
For more information, email us at mail@goodhormonehealth.com