HEAR YE, HEAR YE: Patients Talk about Life with Cushing's

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You can read about Cushing's. This blog and many other websites offer the text version of this devastating disease.

Hearing people who have Cushing's describe their path to diagnosis and treatment is quite emotional, as it fills in the gaps between the written word. I am amazed at how people stumble onto diagnosis after years and years of being ignored by their doctors. Many Cushies visit five to ten doctors before they even see an endocrinologist, then many go to see several "endiots"--our affectionate term for endocrinologists who don't quite understand the way Cushing's presents in today's patients--before they receive the help they deserve.

Take a listen to real Cushing's patients who describe their onset of symptoms and how they fought to get the diagnosis. For anyone fighting this disease, it is truly heartbreaking to listen to how people have struggled and triumphed over this disease. I have yet to listen to one that did not bring tears to my eyes.

To all my fellow Cushing's patients out there who have fought this disease with such determination, courage, and moxie: I salute you. I dedicate all of these efforts to you, to us.

Go to the Cushing's Help and Support website to choose the podcast format of Cushie Chats: BlogTalkRadio or iTunes.

Go directly to Cushing's on BlogTalkRadio.



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DEATH BY CUSHING'S: Accidental death verdict on 40-year-old mum

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It is with a heavy heart that I share this story today about an unnecessary death, due to Cushing's. I send my most sincere condolences to the Richardson family and all those affected by Marie's passing.

This just shows that often MRIs miss teeny tiny tumors. Doctors were unsure if I had a pituitary tumor, although they agreed there was a suspicious area. During surgery, my neurosurgeon easily spotted the camera-shy tumor, which measured 3 millimeters or 0.12 of an inch. That's really small to wreak such havoc on the body. Dastardly little tumor!

If you think you have to waste, you are wrong.
If you don't fight to get yourself diagnosed and treated, then who will?
If not you, then who? If not now, then when?

~Cushie Melissa
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Accidental death verdict on 40-year-old mum

Published Date: 03 July 2009
By Garth ApThomas

A CORONER has recorded a verdict of accidental death at the inquest held into the death of a 40-year-old Wrexham mother.

Marie Richardson, of Bryn Hafod, died in March at the Maelor Hospital.

A post mortem examination found that she had died as a result of a haemorrhage involving the pituitary gland, which plays a key role in the body's hormonal system.

The inquest was told a post mortem examination had been conducted by Dr Anthony Burdge.

Giving evidence, Dr Burdge said that it was probable the bleeding had been caused as a result of thinning of the blood and not a trauma such as a fall.

Contributory factors in Mrs Richardson's death had been Cushing's disease, a very rare condition involving a hormone disorder, and bronchial pneumonia.

The court was told by Mrs Richardson's husband, Andrew, that his wife had started to experience ill health, including swollen legs and constant backache.

Her mobility was badly affected. Mrs Richardson was admitted to the Maelor Hospital.

Consultant physician Dr Stephen Stanaway said that as part of the treatment, Mrs Richardson received a small dose of a blood-thinning drug to help ensure she did not fall victim to clots.

She had been given a scan involving the pituitary and there was no evidence of a tumor.

It transpired the post-mortem had found Mrs Richardson did have a tumor, which had experienced bleeding.

Dr Stanaway said that Mrs Richardson had not liked the scanning process and moved at one point – it was important for patients to remain still.

Acting coroner John Gittins asked if Mrs Richardson would have been administered with blood thinner if the tumour had been known about at the time of treatment.

Dr Stanaway said it would have to be a balanced decision but he felt that she would have been.

Legal representatives for Mrs Richardson's family and the NHS Trust were present at the inquest. Dr Stanaway was asked a series of questions about whether anything further could have been done about Mrs Richardson's treatment while at the Maelor.

He said that with hindsight the only potential other avenue may have been if she had been given steroids.

But Dr Stanaway stressed he doubted this would have been successful, emphasizing Mrs Richardson was a very poorly woman and it would be impossible to say that administering steroids would have saved her.

Recording his verdict of accidental death Mr Gittins emphasized: "This is not an indication of responsibility, blame or judgment.
"That is not my jurisdiction.

My very sincere condolences go to the family."

Page 1 of 1

* Last Updated: 03 July 2009 8:35 AM
* Location: Wrexham

DEPRESSED: Withdrawal from steroid cortisol replacement

My body is not making any cortisol on its own. Cushing's disease made my hypothalamus-pituitary-adrenal axis off kilter. Now, I am taking steroid hormone replacement in pill form--hydrocortisone--to restore the proper amount of cortisol my body stopped making once the benign pituitary ACTH-secreting tumor was removed.

I have been feeling all of the expected symptoms that are related to dropping my dose of hydrocortisone weekly.

* severe fatigue
* dizziness
* nausea
* headache
* joint aches
* back aches
* depression

Lying down and closing my eyes gets me through the first three. Darvocet, my pain killer friend, gets me through the aching pain. The last one bothers me more than the others. Even taking 200 mg of Sertaline/Zoloft, an antidepressant, doesn't help me combat the depression brought on by low cortisone. Even a friend said she couldn't cry at all on 100 mg--it helped her that much. I'm on double and it's like I'm not on anything.

This got me thinking of the mind bender that is as debatable as the Chicken v. Egg case.

If a tree falls in the forest and no one is around to hear it, does it make a sound?

I am wondering if the same is true about me and my recovery.

It is day 24 after pituitary brain surgery.
Yes, they went through a natural orifice to access the part that hangs from the brain. The docs didn't do a craniotomy. They did not shave my head, and I did not lose my hair. For that, I'm thankful.
However, they did get pretty darn close to my brain... like a centimeter or so. They gave it a little tickle. I've never liked being tickled.

So, I must ask:

If a Cushing's patient struggles after surgery and no one asks her, is she really even sick?

~ Cushie Melissa

P.S. I admit a large degree of self-pity in this post. I feel needy and disappointed and unaccomplished and helpless. I am really even reluctant to post this now, and you may come back later and this post will be gone. If I can publically confess and own my uncomfortable feelings of being a weak, sick patient, then this post will remain here for others to read.

I post my true feelings because that is what I promised myself I would do. Strong or weak. Good or bad. Happy or sad.

I want to show those fighting with Cushing's that coping with this "rare" disease is difficult. People do not understand. It is not just you, your family, your friends. It happens to all of us. I understand.

So let's know that this is the case.
Let's keep moving ourselves forward.
It makes no sense to wallow.
I hate to wallow.
I do wallow, but I don't like to.
When I get sick of myself, I stop.
Like now.
I'm done.
Happy birthday, America.

:)

CARDIAC PADDLE TO THE CHEST: A Letter from the Founder of the Pituitary Network Association

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I have found the Pituitary Network Association to be an incredibly helpful resource. For the undiagnosed, take this letter from its founder as a cardiac paddle to the chest. For the friends and family of the sick, understand the severity of this disease. It matters not whether you have heard about it before or not.

From Our Founder:

Greetings,

If you are just learning about pituitary tumors and the many associated problems and hormonal disorders let us start with a frank discussion of where you are: Pituitary Tumors, though described in some textbooks as "benign" can be very aggressive and can do irreparable harm. Some can even be lethal IF NOT PROPERLY TREATED!

Do not allow your physicians, or yourself or family to treat these tumors lightly or in a start and stop fashion. Get highly qualified professional help. As with cancer or heart disease, you need expert help, only. The last sixty years have been remarkable in pituitary endocrinology. In the 1930's for instance, Cushing's Disease was a death sentence; patients lived an average of 4.7 years after presentation of illness. In the 1950's, the five year survival rate was 50%. The cure rate for microadenomas today is approximately 90% and improving. Experts in Neurosurgery and Pituitary Endocrinology have so improved the cure rate in a mere 60 plus years that the general medical community, and certainly insurance companies and others, tend to think of Pituitary Disease the way they think of Polio. The threat is over and the scourge has left us. Sadly, with Pituitary Tumors this is not the case. Each and every patient has to be evaluated and "fought for" or the battle may be lost.

These tumors act and grow differently in every patient. A correct diagnosis and treatment plan is essential for the survival of many of us. Yes, clearly, in many they are slow growing and may cause little apparent outward damage. Regretably, many with so called "incidental" or "non-secreting" tumors often have symptoms which their physicians simply forget or are untrained to ask about. Sexual function, mental health, overall ability to function and fit in, are all vital aspects of our lives. The tumor may not "take" our life but may in fact change it to the point of lowering the patient and his or her family into a "living hell". Study, learn, seek expert help and join the PNA as we continue to bring you the REAL facts about these insidious diseases and their often fateful consequences.

We do not mean to alarm you, but in the past twelve years we have witnessed enough deaths and suffering to know with certainty that no one has the right to deceive you about the issues you face. We do not yet have ALL the facts, nor are we here to console you. We are here to inform and alert you about Pituitary and Hormonal disorders. If we help save your life and make it better, we have succeeded.

Good luck and God Bless.

Robert Knutzen, founder
Pituitary Network Association

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CUSHING'S: For the new folks

Hi everyone. I am working on a few blog posts that I hope I can post soon. For today, I'd like to spend a minute or two helping some new folks figure out what to do next. I know I've got a lot of information on this site. Sometimes, you need the basics. Here are the basics. I hope it helps.

HIGH UFCS
A high urinary free cortisol test, or UFC, is the "gold" standard of Cushing's tests. That means, if your UFC value is high, it is likely you have Cushing's. Doctors of all disciplines don't know much about Cushing's, but they seem to remember one fact about Cushing's: you have to have a high UFC to have it.

Now, if you do not have a high UFC, you could still have Cushing's. I took over 15 UFC tests and I never had an abnormal UFC. Never. I was told by eight endocrinologists that I did not have Cushing's. In fact, many patients will never have a high UFC, but many are later diagnosed with cyclical Cushing's. They--and I--still have Cushing's.

The important thing to remember is that hypercortisolism, or the overproduction of cortisol, can be measured in a few different tests. No one tests excludes the diagnosis of Cushing's. However, abnormal test after abnormal tests, in conjuction with multiple symptoms, does point to Cushing's.

midnight cortisol saliva
8 am cortisol serum blood test
4 pm cortisol serum blood test
midnight cortisol serum blood test
24-hour urine free cortisol test (UFC)

High ACTH is indicative of hypercortisolism, since ACTH is the pituitary hormone that triggers the adrenals to produce cortisol.

8 am ACTH plasma blood test
4 pm ACTH plasma blood test
midnight ACTH plasma blood test

Get a good understanding of Cushing's, ACTH, and cortisol, according to links on Labtestsonline.org. Be sure to click on all five tabs above each entry:

ACTH (adrenocorticotropic hormone)

cortisol

Cushing's 1 Cushing's 2 Cushing's 3

From labtestsonline.org:

Is there anything else I should know?

How is it used?
ACTH levels in the blood are measured to help detect, diagnose, and monitor conditions associated with excessive or deficient cortisol in the body. These conditions include:
* Cushing’s disease: excess cortisol that is due to an ACTH-producing tumor in the pituitary gland (usually a benign tumor)
* Cushing's syndrome: refers to the symptoms and signs associated with excess exposure to cortisol. In addition to Cushing’s disease, Cushing’s syndrome may be due to an adrenal tumor, adrenal hyperplasia, the use of steroid medications, or due to an ACTH-producing tumor that is located outside the pituitary (such as in the lungs).
* Addison's disease, primary adrenal insufficiency: decreased cortisol production due to adrenal gland damage
* Secondary adrenal insufficiency: decreased cortisol production because of pituitary dysfunction
* Hypotituitarism: pituitary dysfunction or damage that leads to decreased (or no) hormone production by the pituitary – including ACTH production

Measuring both ACTH and cortisol can help to differentiate among some of these conditions. Because the level of ACTH normally changes in the opposite direction to the level of cortisol, your doctor can learn much by identifying an imbalance in this relationship and the direction in which the imbalance occurs. The table below indicates the common patterns of ACTH and cortisol seen with different diseases involving the adrenal and pituitary glands.

Cushing's disease (pituitary tumor making ACTH)
High Cortisol
High ACTH

Adrenal tumor
High Cortisol
Low ACTH

"Ectopic" ACTH (ACTH made by a tumor outside the pituitary, usually in the lung
High Cortisol
High ACTH

Addison's disease (adrenal damage)
Low Cortisol
High ACTH

Hypopituitarism
Low Cortisol
Low ACTH


OTHER HORMONES
The pituitary is the master gland. It controls every hormone produced in the body. The presence of a pituitary tumor will press on cells it shouldn't press on up there, causing the malfunction of other pituitary hormones. Therefore, if you are to test if the pituitary is affected by a benign tumor or microadenoma, it is important to test other hormone levels.

Oregon Health and Science University's Pituitary Unit: The Basic Pituitary Disease / Pituitary Tumor Work-up and A Clinician's Guide to the Work-up of Pituitary Disorders

Here are a few other sites that discuss the testing process. You will find that many repeat the same info. That is good. This repetition gives you the sense that there is an agreed protocal on how to diagnose this disease. Again, getting a high UFC is most of the battle. Once you have a high UFC test in your pocket, you can pretty much get doctors to order the other tests without much hassle, or so I've been told.

Cushing's Support & Research Foundation's Fact Sheet and Cushing's Support & Research Foundation's Diagnostic Testing for Cushing's Syndrome

Pituitary Network Association's Cushing's page.

This is a little more complicated reading from a medical journal, but again, I think you can work your way through it: The Diagnosis of
Cushing’s Syndrome: An Endocrine Society Clinical Practice Guideline

PNA Disorders Section - Introduction to the Pituitary Gland
Categories:

Our thanks to Daniel Kelly, M.D. (Director, Neuro-Endocrine Tumor Center - John Wayne Cancer Institute at Saint’s John Health Center, Santa Monica, CA) and Pejman Cohan, M.D. (Director, Specialized Endocrine Care Center - Beverly Hills, CA) for providing the following disorder information.

Introduction To The Pituitary Gland

The pituitary is a small, bean-shaped gland located below the brain in the skull base, in an area called the pituitary fossa or sella turcica. The gland is regulated by a region of the brain called the hypothalamus and they are connected by a thin delicate vascular connection called the pituitary stalk or infundibulum. Weighing less than one gram and measuring a centimeter in width, the pituitary gland is often called the "master gland" since it controls the secretion of the body’s hormones. These substances when released by the pituitary into the blood stream have a dramatic and broad range of effects on growth and development, sexuality and reproductive function, metabolism, the response to stress and overall quality of life. The pituitary gland is thus at the anatomical and functional crossroads of the brain, mind and body.

Structurally, the pituitary gland is divided into a larger anterior region (adenohypophysis) and a smaller posterior region (neurohypophysis). Directly above the pituitary gland are the crossing fibers of the optic nerves called the optic chiasm as well as the optic nerves as they project to the eyes. On each side of the pituitary gland is the cavernous sinus which is a venous channel through which runs the large carotid arteries that carry blood to the brain, and important nerves that control eye movements and facial sensation. Because of the close proximity of the pituitary gland to these major intracranial nerves and blood vessels, as well as the vital hormonal control the pituitary gland provides, disorders of the pituitary can cause a wide spectrum of symptoms, both hormonal and neurological.

Listed below are the specific hormones produced by the pituitary:

Growth Hormone (GH): This is the principal hormone that, among many other functions, regulates body and brain development, bone maturation, metabolism and is essential for healthy muscles.

Luteinizing Hormone (LH) and Follicle Stimulating Hormone (FSH): These hormones control the production of sex hormones (estrogen and testosterone) as well as sperm and egg maturation and release.

Prolactin (PRL): This hormone stimulates secretion of breast milk.

Thyroid Stimulating Hormone (TSH): This hormone stimulates the thyroid gland to release thyroid hormones. Thyroid hormones control basal metabolic rate and play an important role in growth and maturation. Thyroid hormones affect almost every organ in the body.

Adrenocorticotropic Hormone (ACTH): This hormone triggers the adrenal glands (located above the kidneys) to release the hormone cortisol which in turn, regulates carbohydrate, fat, and protein metabolism and is essential in the stress response.

Vasopressin - Also called anti-diuretic hormone (ADH): This hormone promotes water to be reabsorbed by the kidneys and is thus essential in water and electrolyte balance.

In disease states, the pituitary gland may under- or over-produce hormones. Decreased or absent hormone production from the pituitary gland is called hypopituitarism (Pituitary Failure). The symptoms and treatments for pituitary failure are listed below:

Hormone
Deficient Symptoms
Treatment

Growth Hormone
Children: Growth delay
Adults: Decreased muscle mass, increased body fat, elevated cholesterol, low bone density (osteoporosis), impaired psychological well-being, poor quality of life Recombinant Human Growth Hormone- Given once daily as an injection under the skin

LH / FSH
Decreased libido, erectile dysfunction, irregular or absent menses, decreased body hair, decreased muscle strength, hot flashes, mood changes
Men: Testosterone- Given as either topical gel or patch or injections
Women: Estrogen + Progesterone-Given as either topical patch or pills

ACTH
Poor appetite, nausea, weakness, vomiting, low blood sugar, low blood pressure, dizziness, body aches Hydrocortisone or Prednisone-Given as daily pills

TSH
Fatigue, weakness, cold intolerance, dry skin, constipation, heavy/painful menses, weight gain, memory loss, mood disturbance
Levothyroxine – Given as daily pills (some examples include Synthroid or Levoxyl or Levothroid or Armour Thyroid)

Prolactin
Inability to lactate
No treatment available

Vasopressin (ADH)
Increased thirst and frequent urination
DDAVP- Given either as daily pills or nasal spray

Pituitary tumors (also called pituitary adenomas) can result in hormonal overproduction causing serious endocrine disturbances such as acromegaly (excess GH), Cushing’s disease (excess ACTH) or prolactinoma (excess prolactin). Other pituitary adenomas are non-functional or "endocrine-inactive," meaning that they do not produce excess hormones. Instead, as these tumors enlarge, they can cause compression of the normal pituitary gland leading to decreased or absent hormone production (hypopituitarism or pituitary failure), visual loss from optic chiasm or optic nerve compression and headaches. Pituitary failure may also result from bleeding into a pituitary tumor, pituitary or intracranial surgery, radiation therapy to the pituitary or head trauma. Other tumors that arise near the pituitary gland which can also impact pituitary hormonal function include Rathke’s cleft cysts, craniopharyngiomas, meningiomas, chordomas, gliomas and epidermoid cysts.

For more quick info, check out the info posted on the left side bar.

I hope this helps.
Cushie Melissa

CUSHING'S DISEASE ENDS MODEL'S CAREER; SHE FINDS BETTER ONE

I have so much respect for Carol Perkins for sharing her story in Ladies Home Journal, the prominent women's magazine. That takes a lot of courage. Women are five times more likely than men to be affected by Cushing's. It is my strong belief doctors dismiss women in their 20s-50s when they present with weight problems, fatigue, headaches, etc. In addition, while they tend to go to the doctors more often than men, it seems that women are more likely to be passive in dealing with the medical community, particularly male doctors. Aren't we all taught how smart doctors are, and how they went to school for all those years, make all that money now, and therefore, they know what they are speaking about, but not us? Little ol' measly puny us? Who are we to think that we know more? It's instinct. It's guts. That's what.

Women with a rare disease are unfortunately being treated contempt and given pithy advice to exercise and lose weight. Most docs are not too subtle in their suggestions, leaving Cushies feeling demoralized, dejected, and humiliated. This must stop. Thank you, Carol, for helping us get the word out. Kudos to you.

It is important to thank the folks at Ladies Home Journal for publishing this important article. I know that with ever mention of Cushing’s disease, there will be at least one person’s life that is saved, as early diagnosis is key to Cushies making a full recovery (and cutting out unnecessary misery).

Do you want to tell LHJ thank you, too? Follow this link to post a comment on the LHJ website.You have to register, but please do it, if you have an extra moment or two. It will show them how appreciative we are. Do it for the Cushies and the ones who love us.

~Cushie Melissa

Carol Perkins' Pet Project: Home bound with a devastating illness, former fashion model Carol Perkins started designing accessories for dogs and discovered a new career.

By Jenny Miller

Originally published in Ladies' Home Journal, July 2009.

Throughout her 20s and 30s Carol Perkins lived a dream life. She traveled the world as a Ford model and appeared in magazines like Vogue and Harper's Bazaar. In her early 30s she continued modeling for clients like Victoria's Secret and started performing with magicians Penn and Teller. When she wasn't working she was out on the town, at art exhibition openings, theater events, and nightclubs. Home was a loft apartment in Manhattan. "It was a fashionable, fast-paced life," Perkins says. "Definitely glamorous."

Then she got sick. "I started getting dizzy and having severe headaches," she recalls of 1995. Even worse for her career, her weight shot up by 70 pounds. She developed a hump between her shoulders and noticed hair growth on her chin. Doctors prescribed antidepressants, weight-loss pills, and migraine medication, but nothing helped. As the bizarre symptoms intensified, Perkins could no longer work. "I was disfigured and unemployable," she says. "I kind of hunkered down and lived off my savings."

In a short time Perkins had gone from jet-setter to shut-in. Many friends, unable to deal with her illness and physical transformation, stopped coming by. "It was a really dark downward spiral," she remembers. Two things kept her from complete despair: dogs and sewing. Perkins had always loved animals, so much so that she'd once considered going to veterinary school. But her travel-heavy career had made it impossible for her to have a pet. Now homebound, she began pet-sitting for friends and neighbors. "They were my emotional support dogs," she says. "Dogs don't care if you're fat -- they love you unconditionally." Perkins also loved to sew, and she began making robes, sweaters, and dog beds as gifts for her canine guests. "There's such a joy in making something with your hands," she says. "I was really grateful to have a purpose."

A year and a half later, Perkins was finally diagnosed with Cushing's syndrome. In her case the rare illness was caused by a tumor on the pituitary gland, which made her body produce too much cortisol and led to her many strange symptoms. Two weeks later she underwent brain surgery to remove the tumor. When she awoke, "It was like a weight had been lifted," she recalls. "I decided right then that I was going to devote myself to helping animals and people."

Back home, Perkins faced a long recovery as her brain healed and her body chemistry returned to normal. But the animals continued to visit, and she kept up her crafting with a newfound purpose -- to turn her hobby into a career. An opportunity arrived sooner than expected. Just a few months after Perkins's surgery, an editor at a fashion magazine happened to see a dog bed she'd made as a gift for a neighbor's pug. The bed was featured in the magazine's May 1997 issue. Perkins had never sold any of her crafts before, but her company had launched -- whether she was ready or not. "The phones were ringing and buyers from Bloomingdale's were ordering," she says. She scrambled to fill those orders, then decided she'd better figure out how to really start a business. "I was the typical entrepreneur in that I didn't have a clue." Perkins spent most of 1998 and 1999 doing research and development, still filling orders from her kitchen table.

In 2000 she launched her company for real. She cashed in all her assets, traded New York City for Savannah, which was more affordable and closer to the textile mills she needed to work with, and opened up Harry Barker. "All dogs are hairy barkers," Perkins explains. Then she finally adopted a dog of her own, a sheltie-collie mix she named...Harry Barker.

Perkins not only found success in business but she also found love: She met her future husband, David Rawle, on a blind date and at 45 became a first-time bride, moving to Charleston to live with him. Today Perkins, 51, and Rawle, 66, share their home with Harry Barker and Josephine, a briard. Harry Barker, the company, has seven fulltime employees and operates out of a dog-friendly office in Charleston. The pet accessories for dogs (and, in more limited fashion, cats), such as collars and leashes, beds, toys, and treats, are available online at HarryBarker.com and in 3,500 stores internationally.

And Perkins didn't forget that promise she'd made to do good things for people and animals. This year the company launched a line of dog treats and toys that benefits therapy-dog programs at a nearby hospital. Many of Harry Barker's products are hand-packaged by adults with disabilities through a community partnership with the local disabilities board. And a good number of items are earth-friendly -- a recently introduced line of environmentally conscious collars and leashes is made out of recycled plastic bottles. But as gratifying as those do-good initiatives are, says Perkins, the real joy of her new career is all about the dogs. "There's just something about a wagging tail that makes me happy."

Originally published in Ladies' Home Journal, July 2009.

FAQ YOU: MELISSA'S MAIL BAG

I wanted to share some questions I've received from someone who is embarking on her testing and diagnostic journey. For me, it is good to share the bios of other folks because when we share details, we share ourselves. Melissa, like me, planned a wedding and struggled with the stress and LOOK of the wedding. I bailed but she finished it wmth her big wedding. Even a small piece of life like this can bring your closer to people who are like you, know what you've been through, and are therefore the best ones to take care of you as you get ready for surgery.

*******

On Jun 21, 2009, at 11:24 P, Melissa wrote:

Hey Melissa,
I wanted to say thank you so much for your help today. It was nice being able to talk with you about things, and I look forward to talking with you tomorrow. I wanted to give you a general time line of my symptoms, etc. just so it will help you know a little more about my situation. It's easier for me to type it out, I get kind of lost with it all when I talk about it sometimes.

I don't know when all of this actually started, if it is Cushings, but when I was a young girl I was always a good weight. Then in forth grade I gained fifty pounds. (Right before I gained the weight I was diagnosed with asthma, and was put on steroid inhalers- at one point I was on four different inhalers. Often had to take steroid asthma breathing treatments at hospital and eventually had to buy the machine for home. Also went on steroids for bronchitis at least twice a year.) I remember too that I had very large eyebrows, etc. and more hair than normal for that age. My hair changed texture as well, going from very straight to very frizzy. I struggled with my weight in middle school and high school. In college I was able to combine exercise with diet and maintained my weight around a size 14. When I did do a diet, like the Atkins it was successful during college . I worked at Banana Republic in college as the window display designer, and so I was very active with my job.

Once in college, went off inhalers since I wasn't at home. Did fine without them, didn't have asthma or problems like I had in high school. Weight was more easily managed.
(Off inhalers 2001-2005)

Here's my recent timeline:

2004- Size 14 and in college, started having some difficulty with concentrating in class, etc. Neck pain and fatigued more.

2005- Gained 60-70 lbs. between 2004-2005. Didn't change eating habits or lifestyle, tried to exercise more and wasn't able to lose weight. Started having mood swings more often, cried a lot. Then in Fall of 2005 started having headaches and dizziness. Almost passed out twice in classes, and had to have people walk me to a car and take me home. Doctors said it was my asthma, and put me back on inhalers (I don't know why headaches and dizziness would be considered asthma related, but I went ahead and took them. - Stopped them again in 2006) Tried Weight Watchers and Curves to lose weight, not successful.

2005-2006- Planned wedding, anxiety increased. Headaches and dizziness increased. So did fatigue and neck and jaw pain. Blurry vision, fatigued, not able to move items on the floor at work. Got married in 2006 (Fall)...had to quit job in November, would get very sick feeling, sometimes unable to get out of bed. Doctors diagnosed neck pain and headaches as "TMJ." Went to two TMJ specialists who fit me with a brace and did pain injections. Nothing helped the headaches, or pain. I was also starting to get times when my heart would race even though I didn't feel anxious. I would get a tingling or energy feeling in my muscles. Even woke up with heart racing sometimes. Heat and cold sensitive. Went to physical therapy on doctors recommendation for neck pain. Didn't help, went for over four months.

2007- Doctors tested for Lymes and MS, both were negative...they treated me for Lymes ("just in case" since I had been in a Lymes area- Wisconsin) with one month of Doxcycline. It didn't help. Daily pain. Daily headaches. Would feel shaky, and was unable to do even small tasks around the house without pain or fatigue. For about six months even blow drying my hair was hard, would get pain and fatigued doing even that. Went to Mayo Clinic, and they diagnosed it all as Myofascial Pain Syndrome and Migraines. Did not respond to migraine medicine. They also diagnosed my frequent heartburn as Gastritis. Took medicine for the heartburn, which was strong and used for ulcers and gastritis...heartburn didn't respond to the medicine after six months. They also tested my heart since it would race periodically, it checked out ok.

Found a counselor and went for a year, to deal with the chronic pain and with the situation (not having a diagnosis that fit or anything that helped). Taught me breathing techniques to help when anxiety happened.

2008- Tried Atkins diet to lose weight, wasn't successful at losing any weight even after four months. (Husband lost 15) Tried a new general doctor, who said Mayo was wrong, that since I had neck and shoulder pain that the headaches weren't migraines..they were tension headaches. Medicine still didn't help. Still fatigued, would stay up late at night. Had been doing that for a few years, where I am unable to go to sleep until 2 or 3 most nights. I will be fatigued all day, and then have more energy at night. Doctors wanted me tested for Pseudotumor Cerebri, had two spinal taps and went to neuro opthamologist. Did not have pseudo tumor. Went back to general doctor who said it was al probably Fibromyalgia and to just forget the pain, dizziness and fatigue. Insulin levels high but glucose ok. Wanted to put me on Metformin. Thyroid functions all ok.

2009- Tried cutting out most sugar to lower insulin instead of Metformin. Insulin levels are now normal. Have cut out most sugar and have not lost weight. I have cut out pop for three years, still have not lost weight. Eat mostly fruits, veggies, lean proteins, and high fiber foods. Get plenty of water. Exercise is hard, but have tried intermittently to exercise consistently over the past three years...painful and get fatigued very easily. Doesn't help me lose weight either. Went to a headache doctor who told me I have Metabolic Syndrome probably (Even though Cholesterol- ok and triglycerides- ok), wanted me to do to Atkins Diet to lose weight and said all my symptoms would just go away if I did the diet. I told him I'd tried it the year before and couldn't lose weight, plus none of my symptoms went away when I did it. He told me to try it anyways. Went back to family doctor who says it is all because I have low vitamin d and I'm overweight. Tells me since I have missed 7 of 12 periods that I probably have PCOS. I have also had pain behind my eyes and an uneasy feeling behind my eyes. Sometimes see floaters and bright spots, not in combination with a headache. I also often see greyish spots in vision. Kind of like there's a fuzzy wall between me and whatever I'm looking at... Lights at night often have a halo around them.

That's just a general time line, but when I went online to look for things with hormones and headaches Cushings came up. I hadn't heard of it. Then I read your blog and Cushings Help and things I didn't even realize were symptoms were listed. I have most of them... The staying up until 2 or 3 am...energy at night. The stretch marks. (Mine go from silver in the morning to really dark pink and purple at night - or range during the month) I have them all over. (Stomach, back, hips, chest, arms, etc.) My face has gotten more round. I have the hump. Actually quite a nice one which I just thought was normal...! :-) My weight is mostly in my stomach. I also have some hair loss...which my mom kept thinking this was all from my thyroid because of that - but my thyroid is fine. (Hair in tub and on floor, have to dust it off after styling hair) I've missed 7 of my last 12 periods. I have more facial hair than I used to...more on lip, etc. darker. I have the problems with concentration, I had to take an incomplete for the first time this semester (I'm in Grad school online through the Savannah College of Art and Design because it was the only way I could continue my education even though I feel this way). I forget what I'm going to say a lot of the time, and I have a very hard time writing papers for school...it's often hard to put information together.

One main thing you wrote is that you got pain going up the stairs. That really clicked for me, because I have a VERY unusually hard time going up stairs. I know I'm overweight but it's not just being out of breath, I get PAIN. I've tried to tell my doctors that and they tell me it's just because I'm overweight by it hurts in my neck and shoulders and head, as well as my legs, etc. Some times it even feels like a "steam pot" in my head, just like it's going to burst from pain. I get that just when I stand up some times too. Even gardening is hard, I get a lot of pain and very fatigued doing small stuff. I even have the muscle weakness, one thing that I've noticed that's really hard is pulling clothes out of the washer. My arms just kind of give out on me doing that.

Anyways,
Sorry if I've typed too much, it's just easier for me to write this all down for you. I get side tracked easily on the phone or talking with people about all of this because it's been so much.

Do you think with my history that's it's a good idea to test for Cushings? I hope I'm on the right track, but things just seemed to match the symptoms to a T. I read stuff on Cushings Help and I'm like "That's me!" It's weird. I am scared to even hope that it's an answer. I've been through so much. It's been hard being newly married and graduating from college...only to have all of this happen. I really appreciate your help, ideas, etc. I hope I can help you in any way as well. Thank you again and I look forward to talking with you tomorrow.

Melissa :-)

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

My dear sweet Melissa.

I'm not a doctor but I play one on the internet. However, do not substitute my advice for anything that a medically trained professional might give you. Wow. Even for disclaimer purposes, that is difficult for me to say!.

You have done a wonderful thing in capturing your story, your life in writing. You took the time to write it out to me, but you will use this time and time again to tell your Cushing's story to nurses, doctors, surgeons, and future blog readers.

You are on your way to a better health already. You have taken control.

My hunch, like yours, is that you have Cushing's disease. Pituitary Cushing's because of your eye problems and headaches. But interesting to see the mix of synthetic steroids through your time line. Are you on steroids currently? We'll have to see how this plays into things. May have aggravated your pituitary Cushings.

I recommend that you begin to test for Cushing's.
Stage 1: at home or nearby: watch your symptoms and get day time tests done.

Stage 2: travel to Seattle to see a Cushing's specialist for night time testing, which is critical for showing a disruption of diurnal variations.

Do you have good insurance coverage? PPOs create less hassle for the patient, so if possible, switch that over. More expensive, but less hoops to jump. You will find this process is long and difficult anyway. If you can throw a little money at it and make stronger faster better decisions by choosing your own doctors and dropping the need for referrals, then I say do it. We switched from HMO to PPO, and it saved us time. I know it did.

My first recommendation is to call Dr William Ludlam, neuroendocrinologist at the Swedish Hospital, Cherry Hill Campus in Seattle, WA. It may take a few months to get an appointment for Camp Cushie, as he only accepts 3 patients for the week long testing spots. Set the appointment. It is worth it. Traveling there got my my highest highs in midnight cortisol and 4 am ACTHs.

Dr. Ludlam, Bill.Ludlam@swedish.org, 206-320-2800. Email him this time line and tell him of your plans to try local testing before you come out there.

I suggest making a second appointment with a more local doctor in hopes that this doctor will begin ordering some basic Cushing's tests. This will be particularly helpful in catching tests during the day. Then you go on to Ludlam and the Swedish for night time tests, since few facilities and hospitals are set up to accommodate that intensive schedule of blood draws. That office manages that process very well.

When I went to see Dr Ludlam, I went after I had initially seen a local endo. I did not get any high results from day time testing with local yokle. So, I travelled without any high results on Cushings tests. I traveled there with hope and instinct on my side. I got my highs there (4 am ACTH because tumor fires off at that time for most Cushies, they don't know why; and midnight cortisol blood and salivary).
On the plane ride home--after I got my medical records--I couldn't stop smiling.

Set appt with other recommended endocrinologist. He can order cortisol labwork (cortisol blood draws at 8 am and 4 pm on same day). Cortisol is highest at 8 am to wake you up, 4 pm cortisol is roughly half of 8 am, then cortisol levels at midnight--tested in saliva or blood serum--should be 0. Any abnormality in this pattern (also known as circadian rhythm or diurnal variation--look it up) indicates a flaw in the feedback loop of the hypothalamus-pituiatry-adrenal axis, or HPA. This means CUSHINGS.

Always make sure they do cortisol blood draws and ACTH plasma draws at the same time. You need both at each drawing. Then, make sure they process the ACTH according to specifications: click here to learn about all tests

Even if you don't know it all, learn the language. Doctors refute you less if you are knowledgable and speak their language. Read the medical literature. Discuss it with a Cushie friend. You must be able to present your case to doctors who have many other patients to see. It's your one shot to get the right information to them.

A short note about me, I think I've had a milder course of Cushing's since I was 11. That's 25 years. I plan to update my timeline going further back with photos, etc.

So, Melssa and any other newbies reading this. Here are my suggestions based on what worked for me. If you decide to travel to Seattle, there is other info I can post here about where to day for cheap (in the hospital itself--like YMCA) and how to get your records before you leave, etc. I can help, and I want to help. I hope to be online more, so my plan is to answer each of these requests as they come in. Be patient with me because my dear little Elena wants me all the time, and sometime I can entertain her in other ways... but just for short bursts of time.

OK. Sleeping meds are really fully working. Better reread this in the morning to see if I made since! Thank you Ambian 10 mg!

FAQ YOU: MELISSA'S MAIL BAG

From Sweet Sue in Houston, through Facebook:
Melissa, if you can see this, hope you are doing okay with good thoughts and prayers your way. Let us know if you need something.

*******

Hi Sue. Thanks for checking on me. You are so sweet. I am doing well. I am trying to write stuff up to post on my Cushing's Moxie blog. I'm trying to get a follow-up email set up, too. People may be wondering how I am, and I don't want them to worry.

I am doing well, better than expected. I have to remind myself though, it is all synthetic. My body is not making cortisol/steroids on its own, and any good feeling now will soon be replaced with severe withdrawal symptoms as they lower my dose of hydrocortisone week by week.

BUT I am happy.
I feel more in control.
Grateful.
Pensive.
Appreciative.
Wistful.
Optimistic to be moving forward.
Happy to be me.
Happy to have my husband, miracle baby, family, and friends.

It's all good. :)

They must have pushed through an extra dose of clarity with my anesthesia last week.

~Cushie Melissa

BUT YOU DON'T LOOK SICK: The Spoon Theory

You may be dropping by to check on me after my surgery. I am doing well. I'll post more soon.

For now, I want to take a moment to post a story that many Cushing's patients have accepted as their own. We think about spoons and nod our heads in understanding. Even though we didn't author this story, we feel Christine could be writing about our lives.

Please take a few moments to read Christine Miserandino's personal story. It is an eye-opening look into the life of a person with a chronic sickness or disability.

Especially, I want to say thank you to Christine, from all of us out there suffering from a disease that people don't understand.

Read Christine's Spoon Theory here.

I've also posted a Kate's Letter to Friends and Family that may help foster kindness and understanding for Cushing's patients. You can read that previous post on my blog by clicking here

Happy weekend!
~Cushie Melissa

GIVE ME A P: Why the Pituitary Gland Needs a Cheerleader

From the pituitary center at the Oregon Health and Science University. Very helpful info. Details why pituitary patients suffer for too ling and wind up self-diagnosing.

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

How Do I Work With My Doctor To Find Out If I Have A Pituitary Disorder?

In many cases, patients with pituitary disorders actually suffer for years with their condition before it is recognized; all too often, multiple medical specialists fail to recognize the underlying source of their symptoms. This can be an extraordinarily frustrating and costly experience and can leave a patient ill for many years.There are many reasons why the medical community does not easily identify pituitary tumors and pituitary diseases.

First, pituitary disorders are relatively uncommon. This means that primary care providers often do not have the requisite experience to easily recognize these disorders in a clinical setting.

Second, the physical signs and symptoms of pituitary disorders can be common to many different diseases. Together, these obstacles can significantly delay the diagnosis and treatment of pituitary disorders. By searching the internet, reading literature, and talking to other patients, it is sometimes the pituitary disease patient themselves who ultimately identify the pituitary as the source of their problems. Fortunately, once considered, pituitary disorders can be typically diagnosed with a few tests. To aid both patients and their physicians, we have provided a link below to the printable PDF file, "The Basic Pituitary Tumor / Pituitary Disease Work-up." This work up is also outlined in the section, A Clinicians Guide to the Work-up of Pituitary Disorders. The OHSU Pituitary Disease Unit is available to aid in the interpretation of these tests.

For more details on the signs and symptoms of pituitary tumors or pituitary diseases, please see the sections Diseases of the Pituitary -- A Basic Overview or A Clinician's Guide to the Work-up of Pituitary Disorders. (Click here).

Goin' Home

I got released fromthe hospital today around noon. I'm feeling bad but that is a good sign.

We are about 50 miles from the house. We get to see Elena soon! I miss her. I haven't seen her since Thursday morning. It is the longest I have ever gone without seeing her. I can't pick her up because I can't lift more than 10 pounds and she weighs 20! She will just have to be gently placed in my lap. I will take that baby loving any way I can get it. I miss her!

Ok. Gonna go. I'll post more details soon. I am good and happy and SICK.

PITUITARY SURGERY: I Did Survive (to be sung like Gloria Gaynor)

***
Hi everyone. Day 4 in the hospital, and I am doing well. I'm really sleepy, so I will post the highest of highlights, then I'll scoot.

From Neurosurgeon Jukebox Hero:
---NS found a 3 millimeter tumor on the left, just like he saw on both 3T MRIs. My IPSS in November 2007 confirmed the pituitary as the source of excess ACTH production, which is the purpose of the IPSS test. IPSS show laateralization, or which side of the pituitary the tumor is on--only 85% of the time. In my case, my IPSS showed the tumor on the right; but since my NS saw the tumor on the left, just like in the higher powered 3T MRIs. So my IPSS fell in the 15% category of the IPSS failing to show accurate lateralize. The NS located a tumor only on the left. He did explore all around the gland, looking for any other tumors, but he said "my anterior and posterior pituitary lobes were easily separated" and it's why he thinks I didn't face diabetes insipidus or DI. This is different from sugar diabetes. Both the NS and e endocrinologist say that DI usually shows up in the first 48 post op. It is now nearly 72 hours post up. Yay!! Endo did mention that if I started getting up 3 to 4 times a night to urinate, it could be DI. I am supposed to notify her and she will call in the prescription for oral DVAPT.

--- NS did not use a fat plug.

--- NS said there is less than 1% chance that I will develop a CSF leak or cerebral spinal fluid leak.

---he said my tumor was the classic Cushing's tumor. It was encapsulated nicely but when he resected or cut it open, there was a milky liquid inside. It even ran out of tumor some. He scooped out tumor, milky liquid, and a little bit of normal tissue to ensure he got all if the dashardly Cushing's cells. Pathology report is expected within a week or so. Surgeon is confident it was Cushing's aka ACTH-secreting pituitary tumor.

--- he worked with the ENT during surgery. ENT cut through the sinus, then the neurosurgeon punched through the sella turcica on to the pituitary. So, after NS removed tumor, ENT placed stitches to close off incision sites in sinus, then stitched in stints to keep my nose aligned, then placed tampon-like packing into both nostrils to catch blood coming from incision. After only 12 hours post op, the neuro critical care nurse removed the bloody cotton tampon things the next morning after a quick hit of 2mg morphine. Not too bad, really.

---he said be bets I have been educating all the nurses. I told him he was correct. Then he said they should put me out on the conference circuit so I can present my story to educate more doctors. I told him I would love to do that. I see Vanna White booth time and waving in my future!

JFK HAD ADRENAL TROUBLE, TOO!

During our research for Cushing's disease and its post-op counterpart Addison's disease, Jonathan and I have turned the internet upside looking for stuff. Reading reading reading. This was one very interesting historical fact I didn't know.

President John F. Kennedy suffered from Addison's disease, or adrenal insufficiency. The John F. Kennedy Presidential Library includes a four-page summary of his condition and how he handled it as he ran several campaigns.

As an aside, we have been doing family research on ancestry.com. I found a fourth cousin who traced our shared side of the family to the Kennedys. My grandmother and Rose Fitzgerald Kennedy are 8th cousins, and JFK Jr. and I are/were 10th cousins.

Perhaps adrenal issues run in our family....?

I LOVE IT WHEN A PLAN COMES TOGETHER

GETTING THE BALL ROLLING

I have been focusing on pregnancy, baby Elena, and family for 18 months. She is almost 9 months old now. Elena is fantastic beyond measure. She is a wonderfully curious and loving child. She is a very good and happy baby. I am proud to be her mama!
 

 

Raising a baby while having Cushing’s has been rough on Jonathan and me. I do as much as I can, but he picks up the slack, after working his 40+ hours. He has been wonderful to Elena and me. Through this experience, we have grown closer and stronger both as a couple and a family. In the end, I know that while my house is not as clean or organized as I would like, I know that I have done the one job that was most important. I have cared for my precious Elena every day. She is happy and healthy. We are so close. That’s what really matters.

******

ON TO SURGERY

The orginal plan was to nurse Elena for three months, then wait three months to have pituitary surgery in March 2009. However, when the time came, we felt Elena was just too small to wean after only 3 months. We adjusted our schedule. I nursed her for seven months, set up my doctor appointments for late May, and weaned her in April. The goal? Pituitary surgery in early summer 2009. (I mention this here because breastfeeding keeps the pituitary active by producing prolactin, the hormone necessary for milk production.)

On May 27, 2009, we traveled to San Antonio. We met with the endocrinologist who diagnosed my Cushing's in December 2007. She sees that my symptoms have continued and my lab work continues to show pituitary disease (high cortisol, high ACTH, low IGF-1=70, low vitamin D=15, rising a1c=6.1). She is willing to follow me post op—partnering with my local endo at home--and is ready to do it if the neurosurgeon is ready. She trusts him completely, and she sends all of her pituitary cases to him. She tells me he is well known in Texas for taking the difficult surgical cases that others say are inoperable… and he gets it done.

On May 28, 2009, I had a 3T MRI. This was the second time I had an MRI at this facility. I was in the same room as before. After the usual prep, I was lying on my back and they moved me into the long tube machine thing.

I asked, “Are my feet showing?”

Tech said, “Yes.”

I asked, “Is this a 3T MRI machine? I don't think my feet stuck out last time.”

He said, “No.”

I said, “My neurosurgeon ordered a 3T. It’s what he needed last time.”

He hit the button to slide me out of the machine and checked the papers.

He said, “Yes, we need to move you to the other machine. It was written on the second page of the order, and there was a paper clip covering it.”

NOTE: ASK QUESTIONS! YOU KNOW BEST, AND YOU CARE MORE ABOUT YOUR HEALTH THAN ANYONE ELSE!! BE AWARE OF EVERYTHING! DOUBLE CHECK EVERYBODY!

Several techs did tell me that my neurosurgeon is the one of the best there at the hospital, and when the other neurosurgeons need help during surgery, they call my neurosurgeon. =)

Ok, so although we were late after the MRI switcheroo (I had to wait my turn for the machine), we met with my neurosurgeon. Still like him. We really, really like him. This is the same one who ordered the inferior petrosal sinus sampling (diagnostic!) and cleared me for surgery in December 2007. He understood our need to try for a baby, and he was so excited when we showed him a photo of Elena. I felt he was really rooting for us. I explained I was ready to get back on the surgery fast track.

He said he read the MRI films on his computer, consulted with their neuroradiologist, and they see two suspicious areas on my pituitary. One is located on the right side, one is located on the left. He couldn’t say whether they increased in size.

The neurosurgeon says he is ready to go in and get the tumors out. HE CLEARED ME FOR PITUITARY SURGERY.

The best part is that the neurosurgeon said, and I quote, “I don’t see any reason to make you go through the hassle of retesting” since my symptoms, lab work, MRI and IPSS results all point to Cushing’s disease. He said, “We won’t find out anything we don’t already know.” If you are a Cushie reading this, you know what a relief that is. Plop plop fizz fizz!


 

On June 1, 2009, I get the call. Surgery in 10 days!

When: Thursday, June 11th at 2:30 pm (I fast from midnight on!)

What: pituitary surgery (endonasal transphenoidal pituitary resection)

Length: surgery should take 2-3 hours

Where: San Antonio, TX

Hospital stay: 3 to 4 days

Recovery time: THIS WILL NOT BE A QUICK RECOVERY. Doc says we will feel much worse before we feel better. Most patients take 6 to 24 months to feel better. Extended recovery time is not from the surgery but from the hormone replacement process.

Feelings: relief, excitement, anxious. READY.

I know many of you will not be able to contain your outpouring of love and support for me, and you will want to send flowers. PLEASE DO NOT SEND FLOWERS. The surgeon removes the tumor(s) through my nose and sinus cavities. Flowers in the patient’s room are not too friendly to the recovering nose. Don’t worry, an email will do nicely, too.

*****

I’ve added pituitary surgery information a lot of to my Cushing’s website.
You will find all that you ever wanted to know below.

For surgery updates, please check back here often. We will be posting on Facebook, too. We will have proxies post on my wall, so be sure to check there as it won’t show up on my status updates. I will do my best to keep everyone posted. Forgive us if we get a little busy!

So, there you have it. I love that it all worked out according to our plan. I can’t help but smile at my ability to pull all of it off. I have Jonathan, I have Elena, and I have my feet moving in the right direction. All that makes me smile.

ONWARD!
Melissa

A LETTER TO FAMILY AND FRIENDS: WHAT A CUSHING'S PATIENT WANTS TO SAY BUT CAN'T

**

Although I have made many transitions in the past few years (career to no career, single to married, 1st home to 2nd home, childless to mother, healthy to sick), I have really struggled with one aspect of Cushing's disease. To this day, I still can't shake it.

How do I explain

a disease that no one has ever heard of
in a sound bite that will hold the attention of the listener
while being factually accurate and
true to what I am experiencing
while describing the pervasive devastation to every part of my body
but without being Debbie Downer?

I've realized this, for me, has been mission impossible.
I give too many details, and their eyes glaze over.
I lie and say I am fine, or I gloss over many details.

The result?
1) I haven't adequately explained my condition to others.
2) I feel lonely thinking no one understands.


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

So, tonight, the last Saturday before my pituitary surgery, I make my final attempt. I'm trying something different. I'm using someone else words to describe all that I can't.

I present to you an eloquent description of my life as a person with Cushing's disease. Kate is a dear friend who is a Cushing's survivor, advocates for Cushing's awareness, and has been a wonderful leader for all Cushies. She had the courage to capture the patients' experience--and it's different from what you will read on many websites describing this disease. We thank her for that.

Kate is featured in the video clip The Science of Obesity.


I hope you will take a few moments to read Kate's letter.

-------------------------------------------------

Dear friends and family:

I am writing this letter to share with you some basic facts about Cushing's Disease and the recovery process so that you will have sufficient information to form realistic expectations about me and my ability to engage in certain activities in light of this disease and its aftermath.

As you know, Cushing's is a rarely-diagnosed endocrine disorder characterized by hypercortisolism, or the overproduction of cortisol. Cortisol is a hormone produced by the adrenal glands. It is vital to regulate the body's cardiovascular functions and metabolism, to boost the immune system and to fight inflammation. It's most important job is to help the body to respond to stress.

 

The adrenal glands release cortisol in response to stress, so athletes, women experiencing pregnancy, and those suffering from alcoholism, panic disorders and malnutrition naturally have higher-than-normal levels of cortisol.

People with Cushing's Syndrome live life with too much cortisol for their bodies as a result of a hormone-secreting tumor. Mine is located in the pituitary gland. Endogenous hypercortisolism leaves the body in a constant state of "fight or flight," which ravages the body and tears down the body's major systems including cardiovascular, musculoskeletal, endocrine, etc.

Symptoms vary, but the most common symptoms include:

* rapid, unexplained weight gain in the upper body with increased fat around the neck and face ("moon facies")
* buffalo hump
* facial flushing/plethora
* muscle wasting in the arms and legs
* purplish striae (stretch marks) on the abdomen, thighs, buttocks, arms and breasts
* poor wound healing and bruising
* severe fatigue
* depression, anxiety disorders and emotional lability
* cognitive difficulties, including problems with concentration, memory, word recall
* sleep disorders due to abnormally high nighttime cortisol production
* high blood pressure and high blood sugar/diabetes
* edema
* vision problems
* premature osteoporosis
* in women, signs of hyperandrogenism such as menstrual irregularities, infertility, hirsutism, male-patterned balding and steroid-induced acne.


As you can see, the effects of the disease on the body are dramatic. Worse, the psychological and emotional effects of having a chronic, debilitating and disfiguring disease range from distressing to demoralizing.

Imagine that, in the space of a year, you became unrecognizable to those around you and to yourself. You look in the mirror, but the person staring back at you is a stranger. You endure the stares and looks of pity from those who knew you before Cushing's, fully aware that they believe you have "let yourself go" or otherwise allowed this to happen to your body. Nothing you can say or do will persuade them otherwise, so at some point, you stop trying and resolve to live your life in a stranger's body.

You feel increasingly sick, but when you explain your array of symptoms to your doctor, you are dismissed as a depressed hypochondriac who needs to diet and exercise more. Worse, your family members think the same thing — and are often quick to tell you how you need to "change your lifestyle" to overcome the effects of what you eventually will discover, once properly diagnosed, is a serious and rare disease.

If only it were so simple! No one would choose to have Cushing's. Those of us who have it would not wish it even on our worst enemy.

Most people with Cushing's long for the ability to do simple things, like walk a flight of stairs without having to sit for half an hour afterwards, or vacuum the house or even unload a dishwasher.

One of the worst parts about this disease is the crushing fatigue and muscle wasting/weakness which accompanies hypercortisolism. Not only do we become socially isolated because of the virilizing effects of an endocrine tumor, which drastically alters our appearance, but we no longer feel like ourselves with regard to energy. We would love to take a long bike ride, run three miles or go shopping like we used to — activities, which we took for granted before the disease struck. Those activities are sadly impossible at times for those with advanced stages of the disease.

Sometimes, as with any serious illness, performing even basic tasks of daily care such as showering and dressing can exhaust the limited reserves of energy available to a Cushing's patient.

How do we explain to you what it's like to watch our lives slip away? How do we explain to you what it's like to watch your lives move on in fulfilling ways while ours stand still? What response is sufficient to express the grief and frustration over losing so much of ourselves? It is often difficult to find the strength to explain how your well-meaning words of prompting and encouragement (to diet or exercise) only serve to leave us more isolated and feeling alone.

Though we wouldn't want it, we wish our disease were as well-understood as cancer so that those who love us would have a frame of reference for what we go through. With Cushing's, there is such limited public awareness that we are left to describe the effects of the disease from a void, often with limited understanding from those who love us most, which is disheartening.

The most frustrating misconception about this disease is that we somehow are "doing this to ourselves," or delaying recovery because we need to continue steroid replacement or lack the energy to exercise often, which is sadly false. Trust me that we would love to have that much control over such a terrible disease.

Fortunately, there is a good likelihood of remission from Cushing's in the hands of a skilled pituitary surgeon. Unfortunately, the long-term remission rate is only 56%, meaning that 44% of people with Cushing's will require a second (sometimes third) pituitary surgery, radiation or bilateral adrenalectomy to resolve the hypercortisolism. Without successful treatment, Cushing's leads to death. Even with successful treatment, I will have to be monitored for possible recurrence for the rest of my life.

After surgery or other treatment, the recovery period can last months or even years. Because the tumor takes over control of the body's production of cortisol, the adrenal glands, which had lain dormant prior to surgery, require time to start functioning properly again.

Until this happens, we must take synthetic steroids or else risk adrenal insufficiency or adrenal crisis, which can be quickly become life-threatening. Careful monitoring of our cortisol levels is critical during the weaning period. It is a rare but sad fact that some people's adrenal glands never return to normal, and those people must continue to take hydrocortisone or prednisone — sometimes for life — simply in order for the body to perform correctly its basic systemic functions.

The physical recovery from surgery can be quick, but the withdrawal from hydrocortisone can be a lengthy and extremely painful process. As I described above, Cushing's causes a tearing-down of muscles and bone. While there is an over-abundance of cortisol in our bodies (as a result of the tumor), we often can't feel the effects of the muscle-wasting and bone deterioration because of the anti-inflammatory action of cortisol. Upon weaning, however, these become painfully (literally!) evident.

The physical pain experienced while weaning from cortisol has been described as worse than weaning from heroin. When cortisol levels are low, one experiences the symptoms akin to a really bad flu, including severe fatigue ("like a wet cement blanket laid on top of me"); weakness and exhaustion; nausea; headache; vomiting; mental confusion.

It is imperative for people who are on replacement steroids after Cushing's surgery to carry extra Cortef (or injectable Solu-Cortef) with them at all times in addition to wearing a medic alert bracelet so that medical professionals will be alerted to the possibility of adrenal insufficiency in the event of an adrenal crisis.

People who have struggled with Cushing's Syndrome all hope to return to "normal" at some point. Though none of us want to have Cushing's, it is often a relief finally to have a correct diagnosis and treatment plan. For many, there is a gradual resolution of many Cushing's symptoms within a few years of surgery or other successful treatment, and a good quality of life can be achieved.

Regrettably, this is not possible in every case. Depending on the severity of the disease and the length of time before diagnosis and treatment, the prognosis can be poor and lead to shortened life expectancy and diminished quality of life. This is not a choice or something we can control, but it is the reality for some people who have suffered the consequences of long-term hypercortisolism.

The best support you can give someone who is suffering from Cushing's or its aftermath is to BELIEVE them and to understand that they are not manufacturing their illness or prolonging recovery. Ask them what they are able (and not able) to do, and then be prepared to help them in ways that matter — whether that be to bring them a meal or help them to run errands, pick up prescriptions from the pharmacy or clean their house.

Because it's these little everyday tasks, which can fall by the wayside when someone has (or has had) Cushing's, and these are the things we miss the most: doing for ourselves.

Ask us questions about the disease, and then actively listen to what we say. We know you don't know much about Cushing's — even our doctors sometimes lack information about this rare disease. But know we appreciate the interest and will tell you everything you want to know, because those of us who have it necessarily become experts in it just in order to survive.

Thank you for caring about me and for hearing what I am saying in this letter. I know you love me and are concerned about me, and I appreciate that so much. Thank you also for taking the time to read this letter. I look forward to discussing further any questions you might have.

In the meantime, I am attaching a brief article written by a woman who recently was diagnosed with Cushing's. I hope hearing another person's experiences will help you to understand what I'm going through so that when we talk, we will be coming from a similar starting place.


~Kate


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Dear Family & Friends,

Endocrinologists (doctors who specialize in Cushing's Syndrome and its related illnesses and issues) realize the medical aspect and know the damaging effects that Cushing's has on the body. Family and friends see their "Cushie" (affectionate nickname for a person with Cushing's Syndrome) suffering and know they are hurting physically and often times mentally and emotionally. However, understanding the debilitation of Cushing's and how it can affect every aspect of a person's life is "only truly realized by those who have experienced the syndrome."

The CUSH organization, a non-profit organization ran by volunteers who have Cushing's or had Cushing's is furnishing this letter for patients to provide to their family and friends in hopes of providing a better understanding of the syndrome.

Cushing's Help and Support, a non-profit family of websites maintained by MaryO, a pituitary Cushing's survivor, also provides this letter for patients to provide to their family and friends in hopes of providing a better understanding Cushing's and its many aspects.

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We're sorry to hear that your family member or friend has Cushing's Syndrome. Having Cushing's is like a war going on inside of the body. The person may feel better at times then at other times. It's common for a Cushing's patient to have burst of energy and then all of the sudden they become lethargic and don't feel like moving a muscle. There are many symptoms that are associated with Cushing's. They include weight gain, fatigue, muscle weakness, shortness of breath, feeling achy all over, headaches, blurred vision, mood swings, high blood pressure, stretch marks (striae), buffalo hump, diabetes, edema, and the list goes on. Hormones affect every area of the body.

Cushing's patients are in a sense like snowflakes….we all have the same composition, "an illness that is caused by excessive cortisol" However, no two Cushing's patients will be exactly the same. Not everyone who has Cushing's will experience the same symptoms, treatment, or recovery. Because not all "Cushies" have these symptoms, it makes diagnosis even more difficult.

Cushing's causes the physical appearance to take on a different look due to weight gain, hair loss, changes in complexion, etc. This can be very disturbing when looking in the mirror. This can cause the Cushing's patient to withdraw from family and friends. Cushing's can be a very lonely illness. Cushing's patients often feel alone like everyone is against them and that no one understands.

Cushing's is not a prejudice disease, it can affect, young, old, white, black or other ethnic background, male and female, though it is more common in women than men.
Cushing's patients need to be able to take one day at time and learn to listen to their bodies. There will most likely be times when naps are needed during the day and often times they may not be able to sleep at night due to surges of cortisol.

Your Cushie doesn't expect you to understand Cushing's syndrome to the full extent. They DO need you to be there for them and try to understand to the best of your ability what they feel and not give up on them.

Often, a Cushing's patient may tend to be moody and say things that they don't mean. If this should happen with your Cushie, try not to take it personally and know that it's most likely caused by the elevated cortisol and disturbances in other hormone levels caused by the Cushing's and not from the heart or true feelings of your Cushie.

It can be very depressing and frustrating having so many limitations and experience things in life being taken from you. Cushing's patients are sick, not lazy, not hypochondriacs, or even the newer term "Cyberchondriacs". If a Cushing's patient says they don't feel like doing something or they express how bad they feel let them know that you believe them. One of the most frustrating things to someone who is sick is to have those you love not believe you or support you. Telling a Cushie to think positive thoughts will not make him/her well and will just be aggravating.

Testing procedures can be lengthy and this can become frustrating for the patient and family. Often, it takes a while for results to come back and this can be stressful. Don't look too far ahead; just take one day at a time and deal with the situation that is at hand at the present time.

After a diagnosis is made, then it's time for treatment. Surgery is usually the best treatment option for Cushing's that is caused by tumors. Don't be surprised if the surgeon's facility wants to run even more tests or redo some of those that have already been done. Your Cushie may have to travel a great distance to find a surgeon who is trained in these delicate surgeries and who has performed many of them.
Once the diagnosis has been made and treatment has been given, then it's time for the recovery process. Not all patients who have surgery are cured and they have to make a choice along with the advice of their doctor as to what their next treatment option will be.

The recovery from the surgery itself is like any other surgery and will take a few weeks to recover. The recovery process obtained from getting a cure from Cushing's is quiet different from other corrective surgeries.

A Cushing's patient's body has been exposed to excess cortisol, usually for quite a long time, and as a result, it has become accustomed it. When the tumor that has been responsible for the excessive cortisol is removed and the body is no longer getting it, this causes the body to have withdrawal symptoms. Withdrawal can be very difficult, causing an array of symptoms: muscle aches, weakness, bone and joint pain, emotional disturbances, etc. (Please read the "Adrenal Crisis" publication at the end of this letter.)

Thank you for reading this. We hope it will help you to understand a little more about Cushing's and the debilitating affect it can have on a person. Thank you for being there and supporting your Cushie during this time in their life. We realize that when a family member has Cushing's, it not only affects the individual but also other family members and those around them as well. Showing your love and support will encourage a speedy recovery for your Cushie.

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While I started this blog to share my journey with Cushing's with friends and family, the more tangible, concrete, rewarding, and unexpected aspect of creating this blog is the feedback I've received from others... sick and trying to find answers. They thanked me for gathering information in this one spot. To those folks, I say, Thank you for taking the time to read my words.