And It's About Time There Was Some Support For Cushing's!
http://www.sltrib.com/ci_3721550
Article Last Updated: 4/17/2006 11:46 PM
Often misdiagnosed Cushing's syndrome ravages the body, but the hormonal disorder is treatable
By Jennifer Barrett
The Salt Lake Tribune
Salt Lake Tribune
Lee Udall Bennion noticed when her appearance slowly started to change several years ago.
She gained weight that she couldn't seem to lose. Her face became "moon-shaped." Hair grew in the wrong places and disappeared from areas where it belonged. Her hands and arms were easily bruised.
"I just thought I was aging badly and was going to be ugly for the rest of my life," said Bennion, a 50-year-old artist who lives in Spring City.
When other symptoms appeared, though, she knew there was more to it than aging. Her energy level plummeted and her skin began to tear.
She had insomnia and couldn't concentrate.
She even gave up painting.
"That's when I knew something was really wrong," said her husband, Joe, a potter.
Many months later, they got the correct diagnosis. Bennion was suffering from a rare disease called Cushing's, a syndrome that floods the body with the hormone cortisol. After surgery and a difficult recovery period, Bennion is painting again, more than a year after she put down her brushes. She's sharing her story so that other people diagnosed with Cushing's will see there is hope for a happy ending.
"A lot of people who put their stories out there [on the Internet] are chronically ill and don't get better," she said. "It was kind of terrifying to read when you're first swimming around in that pool."
Cushing's syndrome can be caused by several factors, but each has the same result. The body's tissues are exposed to large doses of cortisol for a prolonged period of time. The hormone, which usually helps our bodies respond to stress and relieves pain, instead wreaks havoc.
Bennion's symptoms were common. So are dramatic stretch marks, acne, ruddy complexion, a "buffalo hump" on the back, anxiety, depression and trouble concentrating. Kids who contract the illness put on weight, but stop growing. Men can get erectile dysfunction.
Cushing's can lead to high blood pressure, diabetes and frail bones.
If untreated, it can be fatal.
Despite its dangers, it is easily overlooked.
"It starts very, very subtly," said Jack Wahlen, an endocrinologist at McKay-Dee Hospital in Ogden, who has handled 20 to 25 cases of Cushing's in his 27-year career, including Bennion's.
The syndrome affects about 10 million to 15 million people a year, according to the National Institutes of Health. The vast majority of those cases are caused by taking medications to treat other illnesses. Cases of Cushing's disease caused by tumors, such as Bennion's, are exceedingly rare. No one knows how to prevent it. The fact that it's so uncommon can also make it more difficult to diagnose because most doctors have probably never seen a case outside of a textbook.
"When you have somebody who starts putting on weight and who doesn't feel very well, that's like thousands of other people" who don't have the syndrome, Wahlen said.
Bennion saw at least four doctors who found nothing wrong. It was frustrating. "I am not a hypochondriac. I rarely go to the doctor," she said.
In fact, the Bennions live the kind of life doctors want us all to lead. They live close to the land, eating as much as they can from their own garden. They get plenty of exercise while caring for their animals. They cook healthy, whole foods. And they are generally careful about their health.
Bennion even mixed up a special supplement of raw meat, vitamins and kelp for her "crotchety" old dog when his joints started to ache.
It wasn't until Lee and Joe sent photos to Bennion's sister Sara Udall Henderson, a nurse, that they found out what was really wrong.
"She e-mailed us back and said, 'Why is Lee taking steroids?' " Joe said.
Then Lee's sister recalled caring for a woman who was dying from Cushing's after going too long without a diagnosis.
Within days, Bennion was diagnosed. After more tests to determine the cause - a tumor on her pituitary gland - and doing her own research, she began to hunt for a surgeon who could remove the growth.
She wanted someone trained in a new technique in which a finger-size scope would be threaded up her nose, through the sinuses, and back to the gland to remove the tumor.
"It's pretty amazing surgery to watch," Wahlen said. The tumor can be the size of "two or three grape seeds stuck together.
Before the surgery, Bennion's youngest daughter, Adah, sent her a thousand folded paper cranes from Japan, where Adah was living as an exchange student. According to tradition, the cranes will help a loved one get well. Another daughter, Zina, strung them on the family's Christmas tree.
With the tumor removed, the level of cortisol in Bennion's body plummeted and a year of difficult recovery began. For some patients, recovery is as terrible as the syndrome was. (In Bennion's case, recovery was complicated by recurring infections and the discovery that she has a rare blood-clotting disorder.)
"It's a real roller coaster," Wahlen said. For months or years, cortisol has blunted the joint and muscle pain that people usually feel. "Imagine you shut all of that off, and all of a sudden it all comes back."
The patients are put on calibrated doses of hydrocortisone to slowly reduce the amount of cortisol in their bodies.
Still, Bennion said she felt as if she'd been hit by a truck. At night, she would dream of having broken arms and legs.
Depression is also common. Wahlen remembers a case from his training involving a patient who had the surgery and quickly became so depressed she dove from her bed and out the window of her 14th-floor hospital room.
"Somehow, you just get through it," said Bennion, who returned to activity and riding her horses as soon as she could. In fact, she said the horses were good therapy for her throughout the recovery.
One of the first paintings she completed after her disease shows the "young girl inside" of her with her horse, Tiki. It's titled "Horse Medicine."
Recovery was marked by small milestones. She was thrilled one morning after making pancakes and realizing she had done it from memory, rather than having to consult a recipe. Sleep is no longer a luxury.
Bennion is just now almost back to normal - more than a year after surgery and at least five years after the disease began. She's back to her regular weight, without dieting. She's off the medication. Her curly, thick hair has returned, and the mustache is gone. She's planning a fall river trip with Joe.
"It's another sign of life returning," he said.
Now that Lee looks like her old self, Joe tells her how happy he is to have his "hottie" back.
She's glad to be back, too.
"It's taken me a long time to think of myself as a well person again. I was afraid of getting slam dunked again," she said. "I think I'm over this. If I don't get any better than this, I'll be happy."
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Contact Jennifer Barrett at jbarrett@sltrib.com or 801-257-8611. Send comments to livingeditor@sltrib.com.
Cushing's syndrome is a hormonal disorder caused by prolonged exposure of the body's tissues to high levels of the hormone cortisol. It affects an estimated 10 million to 15 million people each year, mostly between the ages of 20 and 50. It affects women five times more frequently than men. The vast majority of the cases are caused by taking medications to treat other illnesses. Cases caused by tumors are exceedingly rare.
* Symptoms: Weight gain, particularly around the midsection and upper back; severe fatigue and muscle weakness; a rounded "moon" face; facial flushing; fatty "buffalo" hump between shoulders; thinning of arms and legs; stretch marks on the abdomen, thighs, breasts and arms; thin and fragile skin that bruises easily; slow-healing wounds; depression, anxiety and irritability; thicker or more visible body hair; acne; irregular or stopped menstrual periods in women; erectile dysfunction in men; high blood pressure. Cushing's can lead to high blood pressure, diabetes and frail bones. If untreated, it can be fatal.
* Causes: Taking corticosteroids for long periods; a tumor on the pituitary gland (Cushing's disease); a tumor on the adrenal gland; or small tumors throughout the body (ectopic ACTH syndrome), which may be malignant.
* More information: Cushing Help and Support (http://www.cushings-help.com); Lee Udall Bennion's personal Web site, which includes journal entries (http://www.horseshoemountainpottery.com)
