survive the journey: death

Martha, it was way too soon, and we will miss you.

Stacey and Martha had been together for 11 years. During most of those years Martha struggled with IBS and acid reflux. About 6 years ago, they noticed her cheeks were getting "bigger" but her legs and arms were very thin. In fact, until the last 5 years she was a thin/normal woman. Stacey said she would squeeze Martha’s cheeks saying "look at those chipmunk cheeks".

Five years ago her health started dramatically changing. She had an ovarian cyst burst, then after problematic periods she had a fibroid tumor removed and a D&C performed. After that she never had her period. Her GP told her she was going through early menopause. Her strength was never the same. Stacey and Martha thought it was because she was pushing herself too hard with a long commute and getting up very early in the morning.

Over the last two years, Martha had unexplained pain in her liver area and was hospitalized. She was diagnosed with a fatty liver, but they could not determine the actual cause of pain. She was also diagnosed with pre-diabetes, and anemia. Almost exactly two years ago Martha became severely depressed. So severe and very unusual for Martha that she took some time off from work (and unfortunately was never able to return) and was referred to a psychiatrist. As is a common routine for those suffering with Cushing’s, it was a wrong diagnosis with too many meds which masked the real problem.

By this point Martha’s face was very, very swollen. Stacey related to me, “Now I know it was the telltale moon face.” Martha went to her GP with concerns about her face and the doctor gave her lasix because she felt the swelling was due to water retention.

In April of 2007, Martha was admitted to the hospital for severe pain in her feet and numbness from her ankles to her mid-section. Going through her records in the past few days, Stacey found an EKG which was noted as being abnormal. They never informed either Martha or Stacey of those results. No one ever performed a second EKG, and she never saw a cardiologist while in the hospital. There was a doctor there who, in passing, said her problem might be hormonal and she should see an endocrinologist. Two endos later (after multiple misdiagnoses, doctor appointments, refusal for disability, etc.) they finally found Dr. F.

The first time she saw Dr. F. Martha asked him "I'm not going to die from this am I?" Dr. F responded, "We all die someday, but I have not lost a patient yet".

Stacey said, “I don't know how true that is, but it made Martha feel better for the moment. I remember standing at the counter taking care of paperwork, and Martha asked me ‘I'm really sick aren't I?’. My response was ‘Yes, hon, but we're going to get through this’. I so wish I had been right.”

Martha’s biochemical testing with Dr. F did show hypercortisolemia, the cause for Cushing’s. Since her pituitary MRI was not diagnostically clear as a source of the Cushing’s, an IPSS was ordered.

Upon getting the pre-op work ready for her IPSS, Martha had another abnormal EKG and she was sent for a chemically induced stress test. During the stress test, she said she became very, very sleepy and the nurse asked if she was ok. A few days after the test she became very dizzy and briefly passed out in the kitchen but refused to go to the hospital. She was so tired of being poked, prodded and not getting anywhere with any answers.

On October 14, 2008, the day before she was to get the results of the EKG, Martha died. Stacey had talked with her several times throughout the day but when she arrived home from work she found Martha unresponsive.

Stacey related to me, “I tried to give her CPR, but I knew it was too late. I only hope she went gently.”

Stacey left me with these remarks:

I wonder if I should have fought harder for her to go back into the hospital, but if Martha's heart at that point was so weak, she may have died at the hospital rather than at home were she felt safe. Or she would have had tubes sticking out of her everywhere and she had already told me she didn't want that. I just wish there was something I could have done to "fix" her. It's not right such a good, caring soul should have to go so early, and with so much pre-death disharmony in her body.

Martha was such a wonderful person. She took the time to learn a stranger's name and to start a conversation with just about anyone. Martha had not worked for two years and had not been in contact with many of her co-workers during her illness. Even so, many of her friends from work went to her service and shared stories about her. They said she was the one to go to for a laugh, smile or a little "pick-me-up" joke. Although many of them had not even talked with her for two years, they were crying during her service. Martha was a good soul. I'm not sure how else to describe her. I'm going to miss her dearly, and I'm not sure yet how I'm going to get through this.

Those of us in the Cushie community were very concerned for Martha, and we were so glad she had Stacey advocate for her. I just wish we had known them sooner. And I wish her doctors in the past years had looked at her symptoms as a whole, not as separate entities.

For those of you who don’t read the cushings-help.com message boards, I’d like to share a bit more about Martha’s symptoms:

Cardiovascular abnormalities
IBS
Acid reflux
Fluctuating TSH
Moon face
Fatty liver
Striae
Ovarian cyst
Hairline fractures (multiple) of spine and other bones
Elevated blood sugar
Insulin resistance
Unexplained flank pain (very common in the Cushie community)
Mid-section obesity
Thin extremities (although not all Cushie’s have thin extremities)
Early “menopause” (Low estradiol, low FSH, low LH)
Unexplained bruising
Buffalo hump
Supraclavicular fat pads
Unexplained foot and leg pain/numbness
Unexplained fatigue
Low ferritin
Anemia
Low IGF-1

Stacey, I want to convey my deepest sympathies, and thank you so much for sharing Martha’s story with me and allowing me to share it with the rest of the world. My heart hurts at such a loss.

If you are suffering these symptoms, do not give up, do not let one or two or even umpteen doctors brush you off. Pursue and prevail. Advocate for yourself. Seek support. Believe in yourself.

Cardiovascular problems are the number one mortality issue for Cushing's sufferers. In patients with Cushing's syndrome (CS) cardiovascular complications determine a mortality rate four times higher than in an age- and gender-matched population.

For those of you in the medical community, please look deeper at your patients when you see these telltale signs. Martha had a slew of them. Many of us only have some of them. In another post, When the "gold standard" becomes tarnished...., I wrote about a study done by Findling, et al, which points out a huge population where Cushing’s is generally overlooked and the depressing mortality for those same folks. Findling lists many reasons, including a study done by Cartagi, et al, where an extraordinarily large percentage of diabetic patients actually had Cushing’s. It is often too easy to pin a diagnosis of diabetes or hypertension without realizing it is a symptom.

In When "the next episode" takes on new meaning...., I also documented this:

"The features of endogenous hypercortisolism (especially, when mild) are protean and coincide with many common clinical conditions like the dysmetabolic syndrome (1, 2). Screening studies in high-risk populations have discovered unsuspected CS in as many as 2–5% of patients with diabetes mellitus (3–7) and suggest that mild CS is more common than previously appreciated."……Cyclical Cushing's syndrome: an update, the full text article says, "Cyclical Cushing's syndrome is a pattern of hypercortisolism in which the biochemistry of cortisol production fluctuates rhythmically. This syndrome is often associated with fluctuating symptoms and signs. This type of case was initially thought to be rare. It has, however, recently been recognized as occurring much more frequently. The phenomenon is important because it can, if not recognized, lead to errors in diagnosis and differential diagnosis of the syndrome and in assessment of therapeutic outcomes. All of these can have very serious clinical consequences."

To read more:

Testing 101: An introduction
3 Jul 2008 by Robin
Since determining endocrine disorders involves a lot of testing, I thought I would spend some time the next few days talking about some of those tests. With Cushing's Disease, an adenoma can cause hypopituitarism and panyhypopituitarism ...

Testing 101: An introduction, Part II
4 Jul 2008 by Robin
Yesterday I wrote mainly about pituitary tumors and their hormones, and how they were involved in testing. Today, I want to include information about adrenal tumors. The adrenal glands sit on top of the kidneys and are sometimes called ...

Testing 101: Imaging
6 Jul 2008 by Robin
Since there is so much involved in the testing and diagnosis of Cushing's Syndrome/Disease, I thought I'd insert some information on imaging here. Imaging alone cannot diagnose Cushing's. There must be biochemical proof of it, also, ...-

Testing 101: Biochemical analysis
7 Jul 2008 by Robin
There are four FDA-approved labs for testing salivary cortisol (Quest, ACL Labs, Esoterix, and Labcorp), and each uses it's own method with varying ranges. The ranges for Esoterix are below: ...

Testing 101: Testing Growth Hormone Levels in Adults
9 Jul 2008 by Robin
The Growth Hormone Research Society met in 2007 in Australia and penned a consensus statement about the problems, testing, and treatments associated with adult GHD. In their consensus statement, they write: ...

Testing 101: IPSS aka BIPSS
8 Jul 2008 by Robin
An IPSS is a test to sample the amount of ACTH draining into the inferior (vs. the superior) petrosal sinuses from the pituitary. Two catheters (one on each side) are threaded from the groin area up each side of the body to a major vein ...

Dynamic MR Imaging of the Pituitary: Same tools, different technique (but wow, what a difference!)
What is Cushing's Disease?

Clinical Correlates of Major Depression in Cushing's Disease
For more information about Cushing’s, see http://www.cushings-help.com .