STORY BYMaury Brochstein was a veteran marathon runner. The 46-year-old businessman was careful with his diet and often got by on three or four hours of sleep. He thought he was the image of health until a friend asked, "Why is your face swollen? You look like you're on steroids."
Although he had no other symptoms, Brochstein was bothered by the change in his appearance. During an appointment with an endocrinologist, he took out his driver's license and said, "Look at my photo. Now look at my face. I run 50 miles a week and watch what I eat. What is wrong with me?"
Had he not been tenacious, Brochstein might have developed more of the symptoms of Cushing's syndrome, a disease caused because the adrenal glands are over-producing steroids.
Yet, with the excess hormones coursing through his body, Brochstein felt fabulous.
Among the cortisone derivatives in the same biochemical family are sex hormones such as estrogen and testosterone as well as anabolic steroids, the infamous performance enhancers. Prednisone is another type of steroid or cortisone derivative used to treat numerous medical problems.
What if a substance made you feel totally healthy? But then, you began to notice changes, monstrous changes, in your physical appearance. Because the corticosteroids were altering the distribution of fats, your face was becoming jowly, your abdomen was ballooning while your arms and legs wasted to toothpicks. Your skin looked ruddy or darker.
If you were female, your menstrual cycle was irregular and serious facial hair was sprouting. Then, over time, you became exhausted.
Brochstein is similar to the one or two people who—without use of medications—develop symptoms of Cushing's syndrome seen each year by Philip R. Orlander, M.D., professor of endocrinology at The University of Texas Medical School at Houston.
"Cushing's syndrome is caused by an excess amount of glucocorticoids, hormones produced in the adrenal gland, typically derivatives of a cortisone product. The most common way of getting Cushing's syndrome is by taking the medication by mouth for a particular medical problem," Orlander explains.
In exceptional cases like Brochstein's, a person may start to produce too much glucocorticoid because of a tumor of the adrenal gland. Brochstein actually had a tumor on each adrenal gland. One tumor and adrenal gland were removed laparoscopically (through a small abdominal incision) in February 2002.
Because glucocorticoids are essential, removal of both adrenal glands would have resulted in Addison's disease, adrenal insufficiency and a medical emergency requiring immediate replacement of the hormone.
A major concern was whether, once surgeons removed one adrenal, the "sleeping" gland would take over. In his case, it did, right away. Orlander says this process can take a year, "and during this time, the patient will paradoxically need to be on cortisone by mouth."
Naturally-occurring Cushing's syndrome (that has nothing to do with side effects from prescribed steroid medication) is caused by:
Orlander emphasizes that Cushing's syndrome occurring for any reason other than prescribed medication is extremely rare.
Only two people in a million get Cushing's as Brochstein did, but because of the number of patients taking steroids for medical conditions, Cushing's syndrome is an everyday phenomenon.
Orlander may see 15 cases a day. "We use these medications for a multitude of complications and diseases: transplants, asthma, rheumatoid arthritis, allergies, back pain. The list is endless."
The physical changes from Cushing's syndrome though are the same, whether they result from this serious disease or from medication side effects. The increasing use of cortisols for medical problems, particularly with transplant patients, has made the syndrome more common.
Similar symptoms may be due to other causes such as polycystic ovary syndrome, ovarian tumors, congenital adrenal hyperplasia, obesity, excessive alcohol consumption or genetic influences towards a round face, heavy abdomen, high blood pressure and high blood sugar.
Patience and a series of laboratory tests are required to determine whether it is Cushing's syndrome. Brochstein remembered two months of extensive blood and urine tests to finally pinpoint his problem. Because he is athletic, he was fortunate that the extreme bodily changes had been curtailed.
Endocrinologists are the ones who treat Cushing's syndrome, such as Brochstein has. Orlander says that people may develop the syndrome from taking a series of short-term steroid prescriptions: perhaps first for asthma, then a knee problem, followed by a medical problem elsewhere.
Problems are compounded when one patient has steroids prescribed by different physicians and filled by more than one pharmacy.
"We try to avoid the use of excess glucocorticoids to treat diseases," Orlander says. Transplant specialists prescribe combinations of newer immunosuppressive agents to reduce or eliminate the need for glucocorticoids. Other specialists use NSAIDs (non-steroidal anti-inflammatory drugs) or forms of cortisone that are not as readily absorbed into the body, such as inhaled steroids for asthma.
Research is underway to design more specific drugs to enhance immune function, rather than affecting many different functions as cortisone does. The push is to develop oral cortisone derivatives that are not absorbed into the bloodstream for conditions such as inflammatory bowel disease.
Cushing's also contributes to other diseases, such as heart disease, diabetes and bone loss. Brochstein, who has competed in 31 marathons, couldn't wait to hit the trail after the removal of his tumor. Six weeks after his surgery, he went for a run. He immediately noticed his joints were aching, when they should have felt rested and ready to go.
After a bone scan, he discovered he had osteoporosis. He now takes a calcium prescription as well as daily supplements and plans to be back on the road when the bone damage has been repaired. Unfortunately, that won't be for six or seven more years.
Brochstein remains active by cross-training. He bikes, hikes, walks and uses an elliptical trainer, a no-impact exercise machine. He and his doctors credit his conscientious physical regime for his success in combating the effects of cortisone.
Because he successfully battled testicular cancer 17 years ago, his take on Cushing's is practical: "Fortunately, this is a very treatable disease. When someone can turn to a page in a book and say this is what you have, here's what we have to do to treat it, and here's what you have to do to continue leading a healthy life, it's easy from there."
UPDATED: 1-12-2004
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Dr. Philip Orlander is a professor of endocrinology at the UT Medical School.
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Tetanus booster for adults
Tetanus does not result from the rusty nail or whatever created the wound. The danger lies in the bacteria Clostridium tetani that lives in the soil or manure on that nail or gardening tool. When these spores get into a wound—deep or shallow—they can produce a potent toxin. Also called lockjaw, tetanus seriously affects the central nervous system and can be fatal.
Onset of symptoms can occur anytime from three days to three weeks. Call your health care provider if you have an open wound, particularly if:
Adults should have a tetanus booster shot every 10 years, known as the Td vaccine. It is a "2-in-1" vaccine that protects against tetanus and diphtheria. It contains a slightly different dose of diphtheria vaccine than what you received as a child. It can be given to anyone older than 7 years and is injected, usually into the arm.
Instead of the standard Td booster every 10 years, adults between the ages of 19 and 65 should receive Tdap one time in their adulthood to boost the immune system for pertussis, as well as tetanus and diphtheria.
Diphtheria, a contagious bacterial infection created that causes severe inflammation of the throat and larynx and can also affect the whole body. Pertussis or “whooping cough” is a serious bacterial infection that afflicted children and infants before vaccines were available. Adults may be infected later in life as their immunities wane. Neither of these infections are related to tetanus, but both vaccines are compatible and convenient to use with the tetanus booster.
