Facing the Unknown
Like most people, Howard Pearl, 55, of Glencoe, Ill., knew little about the body’s endocrine system. But about 10 years ago, he began a lifelong journey with endocrine disease.
In June 1999, Howard Pearl, a Chicago attorney, had a routine physical examination. A few days later, he was surprised to learn he had diabetes, a chronic disease in which the body does not produce or properly use insulin, a hormone produced by the pancreas. To manage his disease, he takes insulin daily.
First Diabetes, then Cushing'sTwo years later, Howard didn’t feel well, and became progressively worse. “I was exhausted but didn’t feel better after sleeping,” Howard said. “I also felt strong urges to eat, even when I wasn’t hungry, and developed a distended belly.”
The latter symptom helped Howard identify his problem. He found a book about women’s weight gain after age 40. “I learned excess cortisol was a possible explanation for a belly like mine,” he said. “I also researched this hormone online and then asked my internist to test my cortisol levels. They were above normal. From my research, I knew I had most of the symptoms of Cushing’s syndrome,” he said.
Cushing’s syndrome is caused by prolonged exposure of the body’s tissues to high levels of cortisol. Cortisol helps the body respond to stress, and maintains the immune system, blood pressure and cardiovascular function. It also converts fat, carbohydrates and proteins into energy.
Cortisol is produced by the adrenal glands. The body carefully controls cortisol production. When cortisol levels are low, the hypothalamus, a gland in the lower portion of the brain, produces a hormone to stimulate the pituitary gland. The pituitary then creates a hormone that stimulates the adrenal glands to produce and release cortisol.
Too much cortisol in the blood over a long period can cause physical changes, including weight gain in the neck, face and upper body, red face, easy bruising, purple stretch marks on the abdomen, diabetes, high blood pressure, bone loss, excess facial hair and fewer menstrual periods in women, muscle weakness and infertility. It can also cause depression and affect memory and thinking.
Excess cortisol may be caused by an endocrine tumor or by taking a high level of cortisol-like medication. When Cushing’s syndrome is caused by a tumor in the pituitary gland, it is called Cushing’s disease. About 80 percent of tumors that result in Cushing’s begin in the pituitary. At times, other tumors (usually in the lungs) can cause Cushing’s. In some cases, a tumor of the adrenal glands is the cause.
A Meaningful DiagnosisWhile Howard believed he had Cushing’s, getting a physician to diagnose it was another story. “Then, someone in Chicago referred me to Dr. Findling,” he said.
Medical College of Wisconsin endocrinologist James Findling, MD, FACP, is renowned for his work in Cushing’s syndrome. He sees patients in the Endocrinology Center at Community Memorial Medical Commons in Menomonee Falls. Howard first visited Dr. Findling at another healthcare facility in 2001.
Dr. Findling conducted tests to check Howard’s cortisol levels. The tests included:
- A salivary cortisol test to measure cortisol levels in saliva between 11:00 pm and midnight. People with Cushing’s have an elevated level of cortisol late in the evening. Dr. Findling helped develop this breakthrough test in the 1990s.
- An inferior petrosal sinus sampling (IPSS), which involves collecting blood samples from the sinuses and an arm vein to determine if excess hormone production is related to a pituitary or a non-pituitary source. Dr. Findling developed IPSS in the late 1970s. Today, the test is used nationally.
“We’re known as the Cushing’s experts. Patients from all over the country come here for diagnosis and treatment,” Dr. Findling said.
“Because Cushing’s has so many variable symptoms, it can be difficult to diagnose. It should be considered for patients with uncontrolled diabetes, high blood pressure and patients under age 65 who have osteoporosis.Women with polycystic ovary syndrome (PCOS) should be screened for Cushing’s, because the symptoms are similar to PCOS. It should also be considered in men with hypogonadism (testosterone deficiency) and people with a mass in the adrenal glands.”
Dr. Findling has educated other endocrinologists about the importance of screening patients for Cushing’s. He hopes to continue spreading the word to primary care physicians. “Screening of those at risk needs to be increased,” he said. “We want physicians to do this simple saliva test.”
Next, Thyroid and Pancrease ProblemsDuring Howard’s care, imaging revealed a mass in his thyroid gland and another in his pancreas. The thyroid tumor was cancerous. He had surgery to remove his thyroid gland and 30 percent of his pancreas.
Sometime later, a tumor was found in Howard’s pituitary gland. Believed to be the cause of his Cushing’s, it was surgically removed. A biopsy, however, showed hyperplasia — an enlargement of the pituitary — not a tumor. The source of the Cushing’s wasn’t found, and Howard’s cortisol levels went back up.
“If all else fails and no other source can be found, the last option is to remove the adrenal glands— the source of cortisol production,” Dr. Findling said.
“Before having my adrenal glands removed, I toughed it out for a couple of years, but I couldn’t take the symptoms any more,” Howard said. “I went ‘shopping’ for a surgeon and decided to go with Dr. Klinger.”
Finally, Adrenal Gland SurgeryIn January 2007, Dean Klinger, MD, now with Froedtert & The Medical College of Wisconsin, removed Howard’s adrenal glands laparoscopically.
“Afterward, I felt so much better,” Howard said. “The Cushing’s was gone.”
“There are no FDA-approved drugs to treat Cushing’s,” Dr. Findling said, “but that could change. We are conducting clinical trials now to test a new drug. However, surgery to remove pituitary or adrenal gland tumors remains our most effective weapon today. People with Cushing’s syndrome have a nearly 100 percent cure rate once the tumors are removed. For people with Cushing’s disease, the cure rate is about 80 percent.”
Without his adrenal glands, Howard now has a condition called Addison’s disease, which occurs when the adrenal glands don’t produce enough cortisol. This is the opposite of Cushing’s. Therefore, Howard must take hydrocortisone for the rest of his life to replace the cortisol in his body. Because his thyroid was removed, he also takes thyroid hormone, as well as daily insulin for his diabetes.
One More ConcernIn 2006, an X-ray revealed a mass in Howard’s lung. “Scans have shown the mass has not grown and is likely benign,” Dr. Findling said. “It could be a neuro-endocrine tumor.”
A neuro-endocrine tumor originates from hormoneproducing cells of the body’s neuro-endocrine system, which consists of cells that are a cross between hormone-producing and nerve cells. Neuro-endocrine cells are found throughout the body, and there aremany types of neuro-endocrine tumors.
“Dr. Findling recommended a thoracic surgeon and, because of my endocrine history, an endocrine surgeon,” Howard said. He met with Medical College of Wisconsin surgeons Mario Gasparri, MD, (thoracic), Douglas Evans, MD, (endocrine) and Dr. Findling at the Froedtert & The Medical College of Wisconsin Clinical Cancer Center.
“This collaborative effort reduced what might have been three weeks of appointments with three different doctors to just one meeting,” Howard said.
“I’ve been a patient at other major healthcare institutions across the country, and I’ve never had this experience. Because of my complicated medical history, standard length appointments don’t work well. At the Clinical Cancer Center, the doctors took as much time as necessary not just to review my case, but to explain it.
“Because hormonal problems have implications for so many parts of the body, my care requires a team,” Howard said, “and the team showed up!”
As of late June, Howard’s team was determining if he should have surgery for the lung mass. Howard continues to see Dr. Findling to monitor his endocrine issues.
Medical College of Wisconsin endocrinologists see patients at Froedtert & The Medical College, the Endocrinology Center at Community Memorial Medical Commons, Lincoln Avenue Primary Care Clinic, St. Francis Medical Arts Pavilion and the West Bend Clinic. For more information, call 414-805-3666 or 800-272-3666.
Source: Froedtert Today
Date: September 2009