Sunday, May 1, 2011

Spinning Out of Control

Unexplained symptoms left Shana Leslie feeling like an old woman trapped in a 30-year-old’s body.

By Susan Flynn

Throughout 2007, Shana Leslie* developed acne, experienced increasingly shorter menstrual periods and gained more than 20 pounds, mostly in her midsection. Her friends were not surprised. “I had just turned 30, was in the middle of a divorce and had recently been promoted at work,” says Ms. Leslie. “So everybody told me it was related to stress.”

The explanation seemed reasonable to Ms. Leslie, a single mom raising a young son in northeast Ohio. She bought larger clothes and visited a dermatologist. But by November, she noticed more changes. Hair sprouted on her upper lip and grew farther down her hairline. Ms. Leslie’s periods stopped completely, and her ankles swelled.

“The only other time my ankles had been so swollen was when I was pregnant, so I went to my obstetrician,” she says. After ruling out pregnancy, the doctor sent Ms. Leslie to an internist. Her blood pressure was very high — 170/110.

Like the young woman’s friends, the physician thought stress might be partly to blame, so he prescribed anti-anxiety drugs and medicine to reduce blood pressure. A month later, during a work seminar in North Carolina, the room seemed to spin. “I had heart palpitations, and I felt like I was going to faint,” Ms. Leslie recalls. At the local hospital, emergency room doctors confirmed her blood pressure was high but couldn’t find anything else wrong.

Back home in Ohio, her internist changed Ms. Leslie’s medication, but an adverse reaction landed her in a nearby hospital. She underwent a gamut of tests: a CT scan, an MRI and blood work. Still, physicians found nothing wrong. Ms. Leslie’s frustration grew.

“My body was spinning out of control,” she says. “I was shaking all the time, and I couldn’t sleep.” Bruises began to dot her legs and stretch marks lined her stomach. In February 2008, Ms. Leslie hit an unrelated snag when she lost one of her contact lenses. This hitch was the turning point for her.

She visited an optometrist, who noticed she had ocular hypertension — higher than normal pressure inside the eye. She mentioned her high blood pressure. After informing her that the two were unrelated, the optometrist listened to Ms. Leslie’s whole story. “He said I had to be my own healthcare advocate,” she says. “He really made me start thinking.”

Ms. Leslie turned to the Internet and began researching secondary hypertension, the only clear-cut condition diagnosed so far. A website mentioned that one cause was Cushing’s syndrome, a rare disorder triggered by prolonged exposure to elevated levels of the hormone cortisol. “Everything on the list of symptoms fit me to a T,” she recalls.

She returned to her internist, who ordered a blood test to check her cortisol levels. They were off the chart. An endocrinologist ran two additional screening tests for Cushing’s. Both showed high cortisol levels. The endocrinologist referred Ms. Leslie to Amir H. ­Hamrahian, MD, an endocrinologist at Cleveland Clinic with expertise in pituitary disorders. “At this point, I couldn’t walk up the stairs,” says Ms. Leslie. “I felt like an old woman stuck in a 30-year-old’s body.”

Dr. Hamrahian recalls first meeting Ms. Leslie, who showed him photos of her thin and vibrant self from just a year before. “You couldn’t recognize the same person,” he says. “She was really in need of help.” Cushing’s syndrome can be tricky to diagnose because weight gain and anxiety are common. Depression, obesity, diabetes and other conditions can also elevate cortisol levels. However, looking at Ms. Leslie’s whole clinical picture, Dr. Hamrahian diagnosed Cushing’s. Excessive cortisol causes skin atrophy, loss of collagen and insulin resistance, the reasons for Ms. Leslie’s stretch marks, bruising and weight gain.

Dr. Hamrahian ordered an MRI to check for tumors on the pituitary gland at the base of the brain. The pituitary controls the amount of cortisol produced by the adrenal gland. Pituitary tumors are the most common cause of Cushing’s. The MRI showed nothing, but he did not give up. He sent his patient to the National Institutes of Health (NIH) in Bethesda, Md., for a procedure called inferior petrosal sinus sampling (IPSS), in which doctors draw blood from the inferior petrosal sinuses, which drain the pituitary. Doctors at NIH compared the levels of adrenocorticotropin hormone, which spurs release of cortisol, from Ms. Leslie’s petrosal sinuses to levels from a vein in her arm. The IPSS revealed higher levels in the sinuses, indicating that a tumor in the pituitary gland was causing her Cushing’s syndrome.

On May 30, 2008, Cleveland Clinic surgeon Robert Weil, MD, of the Neurological Institute and the holder of the Melvin H. Burkhardt Chair in Neuro-Oncology Clinical Research, removed two 3-millimeter tumors. Since then, Ms. Leslie has gradually returned to health. Her cortisol levels and blood pressure are normal, the acne is gone, her menstrual cycle has resumed, and she has lost 40 pounds. “In experienced hands, there is a good chance of cure or long-term remission,” says Dr. ­Hamrahian. “Most symptoms resolve, but patients need to be monitored lifelong for any signs of recurrence.”

Ms. Leslie is grateful for this second chance. “I’m working out, playing with my son and living my life the way someone my age should be,” she says.

* The patient’s name was changed to protect her privacy.