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ANNOUNCER: Being tall is a physical trait that's often valued in our society, whether it's on the fashion runway or basketball court. But for people with acromegaly, excessive growth is one symptom of a serious disease.
ANNE KLIBANSKI, MD: Acromegaly can best be defined as a syndrome where there is excess secretion of growth hormone from the pituitary gland. When growth hormone is secreted in excess, it stimulates the liver to make IGF1, or insulin-like growth factor 1, and this is a substance, then, that acts on the body to stimulate a lot of the glands and a lot of the organs and a lot of the biologic systems in the body that will be affected by too much growth hormone.
ANNOUNCER: In most cases, the excess hormone production is caused by a small non-cancerous tumor in the pituatary gland, which regulates a number of bodily functions.
RENA SELLIN, MD: There are a number of different types of cells in the pituitary, and they interact with other endocrine glands in the body to regulate growth development, reproduction, metabolism, salt and water balance, so they are what we call in endocrinology a "master switch."
ANNOUNCER: In acromegaly, the tumor in the pituatary interferes with the master switch, causing symptoms like the one that gives the disease its name.
RENA SELLIN, MD: It actually derives from the Greek; "acro" means extremities, and "megaly" means big. So the individuals who have acromegaly have large hands, a tall stature.
ANNE KLIBANSKI, MD: It's not at all unusual for hands and feet to grow during normal development or to change a bit with weight gain. In acromegaly, however, you have dramatic enlargements of the hands and the feet, such that the hands become very thickened, and the feet become very thickened as well. Patients sometimes don't experience a change in foot length, but they will experience a change in the width of the foot.
The changes in soft tissue growth or bone growth can also affect other parts of the body, so patients may notice that their jaw becomes bigger, and in fact their teeth may become more widely spaced. Their foreheads may become more prominent, and also, their nose and lips may become broader.
Acromegaly can also lead to high blood pressure, and glucose intolerance, and even frank diabetes.
ANNOUNCER: Acromegaly can be treated when it's caught early, but because many of its symptoms seem unrelated, it often goes undetected.
RENA SELLIN, MD: Some of the early changes somebody might notice is change in their ring size, change in their shoe size. Usually after the age of 18 or 19 our shoe size stays the same, and certainly high school ring, graduation ring, or a wedding ring don't change over time. So if they become too tight, that's one of the early signs.
ANNOUNCER: When acromegaly is suspected, blood tests are usually done to see if the patient has elevated growth hormone levels. These tests, however, may not catch all cases of acromegaly.
ANNE KLIBANSKI, MD: The blood tests are designed to diagnose acromegaly at a time when growth hormone secretion is abnormal, so that it's certainly possible, in very early stages of acromegaly, these tests may be normal. In fact, if there is a great clinical suspicion of acromegaly, and the blood tests that are done for screening purposes are normal, it's important to think about reevaluating these tests after six to twelve months, particularly if these symptoms continue.
ANNOUNCER: When a diagnosis is established, treatment should be started immediately to avoid further complications. Frequently, the first step is to surgically remove the pituitary tumor.
RENA SELLIN, MD: At the time of surgery, the goal in any pituitary tumor, or in any surgeries, to remove the tumor as completely as possible without damaging the normal tissues that are next to it. And that becomes one of the difficulties in that the pituitary, which is a very small and delicate area with a lot of other normal cells next to tumor cells, one tries to remove the tumor cells without damaging the normal tissues. That may not be possible.
ANNOUNCER: When surgery cannot remove the tumor completely, two classes of medications can be used to treat acromegaly-dopamine agonists, and somatostatin analogs.
ANNE KLIBANSKI, MD: The dopamine agonists, bromocriptine and cabergoline-also known as Parlodel and Dostinex-are orally administered, are relatively inexpensive, but they lack efficacy, so that even with cabergoline, the majority of patients cannot be controlled medically. These oral dopamine agonists also have significant side effects, primarily gastrointestinal, when used over time and at high doses.
The somatostatin analogs have the advantage of being more effective so that more than 60% of patients can normalize their hormone levels. They are given by injection, are expensive, and can also cause some, but typically mild, gastrointestinal side effects, including diarrhea and nausea. Overall, the somatostatin analogs have been far more effective than the dopamine agonists in the treatment of acromegaly.
ANNOUNCER: Therapies can stop the progression of acromegaly, and many symptoms go away after surgery or medication. But it's important to understand that treatment has its limits.
ANNE KLIBANSKI, MD: The types of signs and symptoms of acromegaly that are most responsive to normalization of hormone levels are that metabolic ones, such as glucose intolerance or high blood pressure. Also, the soft tissue changes-the changes in the hands, feet, and face-are often likely to respond to medical therapy or surgical cure.
ANNOUNCER: It's also important to realize that eliminating symptoms is not always the same as eliminating the disease.
ANNE KLIBANSKI, MD: Patients with acromegaly are sometimes treated such that their symptoms are better- that is, they feel less tired or weak, and have less sweating. Maybe their hands and feet will not be growing as much, but their hormone levels are not normalized. I think based on what we've learned, it is very critical to try as best as we can to normalize these hormone levels, to reverse the disease as best we can, and also help the patient with acromegaly achieve a normal lifespan.
ANNOUNCER: The only way to avoid permanent complications is to start treating acromegaly early. That's why it's so important to raise awareness of this disease.
RENA SELLIN, MD: If one thinks that their body is changing in the ring size, and the shoe size, or if you see an old friend who looks like Andre the Giant, who was an actor with acromegaly, don't just think to yourself. Tell him. And if they say, "It's none of your business," then, so be it. But if even one of them ends up going to their doctor and finding this problem early, then you've done a good deed for the day.
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