The rare Cushing's disease often causes uncontrollable weight gain but for some, there is a cure.
Cushings disease brought four women together for what they fondly refer to as their “cushie party.”
Jaimie Augustine says, "You don’t gain 70 pounds in 7 months and something not be wrong. I don’t know about you guys, but my family didn’t even believe me.”
Cushing's disease is caused by a rare tumor in the pituitary gland. The tumor itself is not the problem but it causes the body to produce too much of the hormone cortisol. It’s the excess cortisol that wreaks havoc.
"Then I started having hair growth on my chin and on my arms there was excess," Jaimie said. "And, um, I got purple stretch marks on my stomach.”
It didn’t matter if Jaimie ate as little as 1,000 calories a day. She still gained 100 pounds.
It took five years before Jaimie’s doctors pieced together her symptoms and then treated the disease.
Dr. William Ludlam says, "Most centers that deal with this would accept that the first line of treatment is pituitary surgery, brain surgery.”
During surgery, doctors remove the tumor.
“It’s still actually a difficult surgery because these tumors are typically very liquidy, soft tumors and can ooze and go places,” Dr. Ludlam says.
Jaimie’s surgery went well and she’s starting to lose weight.
She says, "I’m from southern California, the place where you have the bleach blondes and the bikini babes and you know I used to think that being skinny was that the end all and be all of everything.”
While she can’t wait to have her body back, Jaimie admits Cushing's has given her a different perspective on life.
Dr. Ludlam is known for solving difficult cases and cautions that the surgery works dramatically for some patients, but not for all.
Fast Facts:Cushing’s syndrome is a rare condition that affects about 10 to 15 out of every million Americans each year.
Most cases are caused by a tumor in the pituitary gland, causing increased production of cortisol, excessive weight gain and a number of other problems.
It takes many patients up to five years to receive a correct diagnosis.
A group of women have started having informal get-togethers they call “cushie parties” to offer support and friendship for one another.
Cushing’s syndrome is a condition that leads to excessive levels of cortisol, a type of hormone, in the blood. It is sometimes called hypercortisolism. The condition is not very common and affects only 10 to 15 out of every million Americans each year. Most patients are between 20 and 50. It is more common in women than in men.
The most common cause of Cushing’s syndrome is a benign tumor in the pituitary gland (a pituitary adenoma). This form of the syndrome is known as Cushing’s disease. The pituitary gland is the master gland of the body. It is responsible for signaling the production of a number of important hormones (like thyroid hormone, cortisol and sex hormones). The pituitary adenoma secretes increased amounts of adrenocorticotropin (ACTH), which causes another set of glands, the adrenal glands, to release excess levels of cortisol into the blood.
As cortisol levels increase, patients begin to notice a variety of symptoms. A key sign of the condition is excessive weight gain, particularly around the midsection of the body, rounded face, and fat deposits on the back of the neck (this is sometimes referred to a buffalo hump). The skin may become fragile and thin and develop purplish stretch marks. Patients bruise easily. Some patients develop bone weakening and may be prone to fractures. Severe fatigue, muscle weakness, high blood pressure, diabetes, depression, and a decrease in both sex drive and fertility may also occur. Women may experience menstrual irregularities and excessive hair growth on the face, neck, chest, abdomen and thighs.
Treating Cushing’s Disease
Once the cause is found, the first line of treatment is usually surgery. The procedure is called a transsphenoidal adenomectomy. Doctors make a tiny incision through the nostril (sometimes they go through the upper lip) to access the pituitary gland. The tumor is located and removed.
William Ludlam, M.D., Ph.D., Endocrinologist with Oregon Health & Science University (OHSU), says that once the source of the cortisol overproduction is removed, levels of the hormone may drop to normal. However, the body becomes used to the high levels of the hormone. So patients must take decreasing replacement doses of the hormone until they reach “normal” production levels.
Transsphenoidal adenomectomy is a very delicate procedure and should only be done by doctors with expertise in the surgery. In experienced hands, about 80 percent of patients are successfully cured. If the surgery doesn’t completely help, doctors may attempt a second transsphenoidal adenomectomy in case any tumor tissue was missed during the first surgery. Some patients require removal of the adrenal gland. Another option is radiation therapy to the pituitary gland. About 40 to 50 percent of adults who receive radiation for pituitary adenomas see an improvement in symptoms.
Living with Cushing’s Disease
Patients with Cushing’s disease often have a hard time getting a diagnosis. The symptoms are often vague and can be associated with other problems or decreasing activity and older age. Ludlam says most patients seek help for five years before they receive a correct diagnosis.
In Portland, OR, a group of women with Cushing’s disease get together from time to time. They call these occasions “Cushie parties.” The women all know the struggles with diagnosis and treatment and have learned to support one another and share their friendships. The women have developed a website to help others around the country. It can be accessed at http://www.cushings-help.com.
The “cushie party” women have developed a Web site to help others around the country.
For general information on Cushing’s syndrome:National Institute of Diabetes and Digestive and Kidney Diseases Web site
Pituitary Network Association Web site
National Institute of Neurological Disorders and Stroke Web site