And It's About Time There Was Some Support For Cushing's!
This is being written for Amber by me, Tami, her partner/girlfriend/life-partner - whatever the politically correct term you want to use. It was 2000 when Amber’s life began to change.
Amber is a paramedic and has worked full-time since 1986 in field of emergency medicine. The job is stressful and requires great concentration and strength and Amber was a natural.
From 1996 to 2000 Amber had severe endometriosis symptoms and in those 4 years had a D&C done, a laproscopic excision of endometrial tissue and took depo lupron injections and depo provera injections. Finally in 2000 Amber chose to undergo a total abdominal hysterectomy. To illustrate Amber’s inner resolve and fitness at that time - within 12 hours of her surgery Amber was walking the halls of the hospital and on one of those hallway walks at hour 20 met her gynecologist coming out of the elevator. Amber asked, jokingly but no jokingly, if she could go home. And within the next hour we were on our way home, less than 24 hours after major abdominal surgery. Amber felt better than she had in a long time for the first few weeks. Her weight upon check in for surgery was 175 lbs, a tiny bit heavy for her 5 ft 5 in frame but she was mostly muscle. She became extremely moody and irritable -very unlike herself.
The next month she started gaining a small amount of weight in her belly and her blood pressure to spike. Then she developed a small hump (buffalo hump) on the back of her neck. She was placed on antihypertensive medication (Hyzaar and Norvasc) and we resolved to eat better. In our minds (and our gynecologists mind also) the weight gain and hypertension was due to the absence of estrogen from the hysterectomy. To us, the hump was actually osteoporosis beginning to weaken her spine and cause a curve - again due to the loss of estrogen. The weight kept coming on slowly and all in her belly. Within a couple more months she was at 215 lbs. Fatigue set in and it was difficult to do anything beyond normal work and housework. Naps became a mandatory daily event. Sometimes she’d wake up from a nap and within 30 minutes be sound asleep again. Again we attributed all this to the loss of estrogen. At the same time her hair lost its luster and began to thin. And she began to have hairy cheeks and chin. Estrogen was to blame we thought. Amber’s cheeks took on a rosy glow and she developed cystic acne. Within a few more months she began to develop weakness in her upper body strength, which even though she was weak she was still very strong for a female due to the demands of being a paramedic.
Our lives went on with Amber compensating as she could for the fatigue. The antihypertensive medication kept her blood pressure under control and the ‘hump’ was not bothersome. During this time she also developed pain and tingling in her right shoulder/arm/hand and was diagnosed with degenerative disc disease and carpal tunnel. She eventually started getting trigger point injections into her neck/shoulder. Eventually she ‘maxed’ out on the injections. After a few more months she was unable to hold an IV needle due to the numbness and was referred to a surgeon for carpal tunnel release. Amber had her carpal tunnel release in 2003. It did not fix the problem.
In December 2004 Amber was at work when she developed horrific right upper quadrant belly pain, shortness of breath, heaviness in her chest, dizziness and weakness. In the ER the only abnormality they could find was slightly low potassium. After IV replacement of the potassium all her symptoms disappeared. A belly CT was done in the ER that day and a small lung nodule was visible on the films. Repeated chest CT’s were done over the next few months and finally a diagnosis of histoplasmosis was made (not unusual due to our location).
It was at this point that Amber began experiencing brain fog. She went from being on top of everything to needing notes placed all over the house/cars/work to remind her to take her medication, wash her hands, empty the coffee pot etc. This symptom was one of her worst and most debilitating. She’d start out on a trip to the store and forget where she was going.
During the next few months Amber had 2 other episodes of the same belly pain and each time her potassium would be low. IV replacements fixed the problem each time. It was during Amber’s ‘yearly’ visit to the gynecologist that we mentioned these three episodes of hypokalemia (low potassium) that we got our first clue of what might be going on. The gynecologist told Amber to have an adrenal study done next time she went to her family doctor (Amber had an appointment scheduled at that time for the next week) as adrenal issues can cause hypokalemia.
Amber’s adrenal study was “normal” and she was placed on KlorCon, a potassium replacement medication. We started noticing that Amber was bruising easily (just leaning against a wall or door jamb would cause a bruise) and healing very slowly. The skin on her hands was thinning and became like crepe paper. Her belly would protrude more at times(she looked about to deliver twin babies) and her cheeks and ears would become beet red and hot to touch. . This usually happened in the early evenings. Her face became round and fleshy. Amber also could not sleep at night and would be up 3 or 4 times to pee during the nighttime.
I had begun researching hypokalemia and adrenal issues and soon I discovered information on Cushings’ Disease. I then recalled a coworker of mine who had had a pituitary tumor removed that caused her some of the same symptoms as Amber was having. Our family doctor begun testing Amber for Cushings’ Disease with a serum cortisol level showing elevated and an elevated 24 hour urinary free cortisol(UFC). JACKPOT!!!
Amber was lucky, her first two tests came back high enough to be referred to an endocrinologist and while awaiting that appointment had 2 additional serum cortisol tests and 2 more UFC ordered by the family doctor. All came back diagnostic for Cushings. The first endocrinologist that Amber could get into didn’t believe that Amber had Cushings. He said she didn’t have the ‘classic’ sign of stria on her belly AND her muscle weakness was not profound enough to be caused by a pituitary tumor. BUT he said he’d order ANOTHER UFC and serum cortisol and we were lucky that he’d just been studying up on Cushings’ Disease because he had to take an recertification test soon. Amber never went back although she did do his testing which all came back elevated.
Onward to endocrinologist number two and the keeper. Dr. S. took one look at her and said, “Honey of course you have Cushings’.” He then ordered pituitary MRI which showed a 5mm x 8 mm tumor!! He referred us to a topnotch neurosurgeon who, after an IPSS indicated which side of the pituitary the tumor was located on, successfully removed the offending tissue. The tissue was stained and was positively identified as the cause of Amber’s high cortisol levels and subsequent signs and symptoms.
Amber’s transphenoidal pituitary resection was performed on December 6th, 2005. In January 2006 it was confirmed that Amber was in remission from Cushing’s Disease. By April 2006 Amber was off the Norvasc (for hypertension) completely and had decreased her dosage of Hyzaar by half. She was still taking a small amount of KlorCon but the dosage was 1/4 of what she had been taking.
It’s been 2 years since surgery and Amber is back to normal. Her hump is much smaller, she no longer bruises easily and heals quickly, the redness is gone from her cheeks and the roundness has disappeared, her head hair is fuller and the facial hair is thinner, her shoulder and neck pains have disappeared and strength has returned to her arms. She got her brain back too, she still uses notes as reminders (but who doesn’t when they get older) but no longer requires them just to function. Her weight is within 15 lbs of her pre-hysterectomy weight and her belly no longer looks pregnant and she is no longer irritable and moody.
Amber’s adrenal glands are still not functioning at 100% (the over production of cortisol by the pituitary tumor causes the adrenal glands to waste away) so Amber is currently taking a small dosage of replacement medication. The endocrinologist expect that soon she will be able to stop the replacements completely.