Day 20: Surgiversary

DATE:  
APRIL 20, 2011.


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


OFFENSE:  

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


ACCOMPLICE:  

ADRENAL GLANDS FOR AIDING AND ABETTING.


STRATEGY:  

TRANSSPHENOIDAL TRANSNASAL RESECTION OF PITUITARY TUMOR.


OUTCOME:  

1) SUCCESS. PATIENT ALIVE. TUMOR REMOVED.


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


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

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

5) PATIENT DECIDES ON NEXT COURSE OF TREATMENT: 

3RD PITUITARY SURGERY vs. BILATERAL ADRENALECTOMY vs. KORLYM

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

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

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

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

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

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

FAQ U: MELISSA'S MAIL BAG

Sue left me a message on my favorite Tips to Get Diagnosed Quickly post. She asks such good questions that I decided to answer them here. Oh, and the babe was sleeping at midnight, so I had time =).

Thanks for the encouragement to be proactive. I have a twenty year old daughter that had a MRI for headaches three months ago. They found an enlarged pituitary gland. Now, three months later it is a recognizable pituitary adenoma 5mm x5mm x8mm. Her doctor keeps reassuring me that it is so tiny that it is probably not the cause of her headaches and that it is no problem that we can't get in to the USC specialist for seven more weeks. I recognize many signs of Cushing's including weight gain in the last six months, new vertical purple stretch marks on her abdomen, complaints of bruises, acne, boils and even an insect bite infected with staph, Also she has constant vaginal yeast infections, a sinus infection diagnosed by the MRI, apathy, and memory problems. My daughter has Asperger Syndrome and she rarely expresses her emotional or physical feelings, so this is somewhat like pieces of a puzzle coming together. Do you or anyone else have advice on waiting to see the so called "best Endocrinologist" as we were referred to by our doc? Do all the endocrinologists mentioned on the site require recommendations from primaries? My PPO does not. Again, thanks for your encouragement to be proactive - when I speak to my daughter's doctor I feel she is trying to assure me it is nothing to worry about. I want to be fully informed and do what is best for my daughter. Thank you for sharing all of your experience so willingly, I am sure you are helping many.

Sue, I'm so glad the words I wrote 18 months ago are helping people still today. I'm sorry your daughter may have Cushing's. No one wants to hear that. This is a big deal. Don't let them minimize it. Be an informed, persistent, and tenacious advocate for your daughter. She is sick and needs your help. Those symptoms sound like Cushing's symptoms. Worry, ok, but pack enough information into your brain that you can nudge them along your way.

One of my favorite posts about the urgency to get treated comes from the former founder of the Pituitary Network Association. I call it, Cardiac Paddles to the Chest .

That pituitary tumor is pretty large. Mine was a microadenoma at only 3 millimeters. A macroadenoma is larger than 10 mm or 1 cm. Both sizes can cause headaches for some patients. Plus, it seems that the pituitary tumors resected during surgery end up larger than the MRI shows.

Do not wait 7 weeks, unless you try all these things:

1. ASK DOCTOR STAFF TO HELP YOU GET AN APPOINTMENT WITH THE SPECIALIST. Ask your doc to call best Endo doc directly for the appt. Staff could try first. Tell them you need their help. Fake apologize for being a worry-wart mom. Go in person, and offer to wait in waiting room until they do it =). Ask that doc 1 fax all clinic notes, labs, and radiology/MRI reports to doc 2 in advance of appt.

2. FIND AN EXPERT. Check these websites for helpful lists of doctors familiar with the treatment of Cushing's. Is there another specialist nearby? Don't put all of your eggs in one doctor basket. Work the system.

* Cushing's Help & Support. On its message boards, we Cushies discuss our favorites under Doctors and Hospitals.

* Pituitary Network Association

* Cushing's Support & Research Foundation

Make an appointment with another doctor if you can get in before 7 wks. Keep other recommended "best endo" appointment. You will feel better for not waiting, and you will arm yourself with more info with a 2nd opinion. Be sure to call your PPO insurance directly to see if doc 2 is in network. Most do not require a referral. Be sure to ask the office staff.

3. TESTING FOR CUSHING'S. Can you tell me what testing your daughter has done for Cushing's? What came back abnormal or high? If no testing besides the MRI was done, ask your referring doc to order some for her while you wait to see the "best endo." Then that doctor can review the results at the first appointment, saving valuable time and giving you a feeling of forward momentum.

Request these tests to diagnose Cushing's
For a good overall assessment of Cushing’s, be sure you leave your doctor's office with:

1) copies of all of your lab results to date;

2) four lab orders for each of the following tests, to test as you see fit, according to your symptoms:

* 24 hour urinary free cortisol (UFCs): normal range is 0-50. diagnostic is over 50.

* midnight salivary cortisol (from your spit): normal range is 0-0.17. diagnostic is 5 and over.

* midnight cortisol serum(from blood draw): normal range is near 0 at this time. suggestive is over 5. diagnostic is 7.5 and over.

* cortisol serum tests (from blood draw). Lab samples must be taken at 8 am and/or 4 pm. If they are not taken at this time, the results do not count.

* random ACTH plasma (from blood draw). Many Cushing's patients see very high levels at 4 am, don't know why. Normal normal range is 5-27. diagnostic is over 48, over 100 really turns the docs on! These lab samples must be handled very carefully to get an accurate reading. I will post a link to the proper handling when I can find one.

In addition, there are many tests that you can ask a doctor to order for an overall hormone assessment. Since the pituitary is the master gland, controlling the release of all hormones in the body, a tumor can cause the malfunction of other hormones produced in other organs.

These tests usually include:

* thyroid panel (TSH, T4, T3)
* pituitary panel (LH, FSH, prolactin, IGF-1 as measured through the liver from growth hormone in the pituitary)
* hormone panel (estradiol, testerosterone, DHEA, etc)
* vitamin B12, vitamin D, ferritin — many Cushies have very low levels of these three. If ferritin is low, it explains hair loss.

Oregon Health and Science Center in Portland, OR shares these suggestions when working with your doctors.

Others may suggest that you ask for other tests. I didn’t feel comfortable asking for specialized tests before I got the basics ones first. I felt that if I had the first battery of tests done and something came back abnormal, then I would ask for others. This approach worked well for me.

4. GET SMARTER. Just like in chess and billiards, winners have several moves in mind before making their current move. Adopt the same concept. Use the wait time to research Cushing's and the next steps. For example, do they recommend an IPSS, which confirms the pituitary as the source of ACTH excess? The test is invasive but proves the tumor needs to come out and it is not just an incidentaloma (real word!). Many patients I know get this test. It is important info for the endo and neurosurgeon, but for you, your daughter, and your peace of mind. My IPSS results were 78 times the normal limit (3 times normal is diagnostic), and this test convinced my neurosurgeon to move quickly.


If I can help, please contact me again. I am trying to make more time for this blog and the people who find me--out in cyberspace. I take my role as advocate seriously.

Peace, joy, and hope to you and your family.
--moxie melissa

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