What Is Acromegaly? 

    Acromegaly is a rare disease that affects about 50-70 out of every million

    people in the U.S. The term "acromegaly" comes from the Greek words "acro"

  (extremities) and "megaly" (enlargement). As the name suggests, acromegaly

   can be recognized by abnormal growth of the hands and feet, as well as other 

   signs such as enlargement of the facial features. However, acromegaly also 

   involves changes in the way the body functions. Over time these changes can

   cause complications that can be life threatening. These health risks make it

   important to attain and maintain control of the condition once it has been

    diagnosed. 

  Acromegaly is caused by the uncontrolled hypersecretion of growth hormone (GH)

 by the pituitary, a tiny gland — about the size of a pea –– located just below

 your brain, behind your eyes. The pituitary is also known as the "master gland"

 — this is because it controls multiple functions, including normal growth,

 metabolism, and reproductive activity. In most cases, the cause of this excessive

 production of GH is a noncancerous tumor on the pituitary. This pituitary tumor

 secretes a high level of GH, causing the enlargement of many body parts, such as

 the hands and feet. Because of where it is located, this tumor can also cause

 headaches with pressure behind the eyes.

Understanding the Causes of Acromegaly

 
There are 3 important hormones that circulate throughout your body that regulate

 many of the most basic activities of your body. These hormones play a key role 

in acromegaly:

 Growth Hormone (GH) - The pituitary releases GH in short spurts throughout the 

day and night, resulting in constantly varying GH levels. When a person has

 acromegaly, abnormally high levels of GH are released by the pituitary. Too much

 GH causes changes in physical characteristics and other aspects of your body.

Insulin-like Growth Factor-1 (IGF-1) - IGF-1 levels rise whenever GH levels rise;

 however, IGF-1 (made mostly in the liver) is released more evenly than GH, and 

IGF-1 levels remain higher longer. When both of these levels are elevated together,

 they can also cause many of the signs and symptoms of acromegaly.

Somatostatin - One of the main effects of somatostatin is its ability to control

 the amount of GH in the body. Researchers have taken advantage of this knowledge

 in developing treatments for acromegaly.
 GH is produced by the pituitary. IGF-1 is produced primarily by the liver in

 response to GH. Somatostatin is produced by the hypothalamus, a part of the

 brain that influences the pituitary.

Possible Signs and Symptoms of Acromegaly

 
Symptoms caused by the tumor:

Headaches
Visual problems
 
Symptoms caused by GH oversecretion:
Hypertension (high blood pressure) or other heart-related problems

Diabetes

Sleep apnea

Excessive sweating

Joint pains

Enlargement of hands and feet

Swelling of soft tissue
 

Other signs and symptoms of acromegaly:

Carpal tunnel syndrome

Skin tags

Menstrual cycle disturbances/impotence

Goiter (enlargement of the thyroid gland)

Sinus congestion

Skin changes, including thickening, oiliness, acne

Coarsening of facial features, including forehead, nose, lips, tongue, jaw.

 Diagnosing and Monitoring Acromegaly

    Several tests are useful in diagnosing and monitoring acromegaly. The most

 important are laboratory tests that measure the levels of growth hormone (GH)

 in the blood. Because these levels vary naturally depending on factors such as

 your age, the time of day, and when you last ate, there are two main approaches

 to measuring your GH.
Random GH testing. To compensate for the variation in levels, your doctor may

 average the values from a series of "random" blood tests.
Oral glucose tolerance test (OGTT). Alternatively, a way to get an accurate

 reading in a single measurement is to take the blood sample after you do an 

overnight fast, followed by an early morning drink of concentrated glucose solution.
Additionally, insulin-like growth factor-1 (IGF-1) levels can be measured, as

 acromegaly in the absence of elevated IGF-1 levels is rare. These levels vary 

by age and gender and need to be adjusted accordingly. The test for IGF-1 levels

 is a blood test.
Blood test for IGF-1 levels. Because IGF-1 is released more evenly than GH, and 

these levels remain higher longer, IGF-1 levels can be taken at any time, with a

 blood test.
Normal GH Levels
Normal GH levels vary, depending on when the measurement is taken:
After an OGTT, GH levels should be
<1 ng/mL.
Without an OGTT, an average level of <2.5 ng/mL from multiple random samples is 

considered normal.
Normal IGF-1 Levels
Normal IGF-1 levels depend on your age and gender, and the reference numbers may 

vary slightly depending on which laboratory provides the results.

Levels of GH and IGF-1 are often measured in ng/mL, which stands for nanograms 

per milliliter. The reference numbers can be slightly different depending on the 

assays used for testing and on the laboratory your healthcare professional uses. 

Please talk to your healthcare professional about your test results and what they

 mean for you. 

 Tests for Acromegaly Diagnosis
 
After acromegaly has been diagnosed, other tests, such as head scans with magnetic

 resonance imaging (MRI) or computed tomography (CT), are designed to look for a 

pituitary growth or tumor, the most likely source of the excessive GH secretion.

 Normally, these tests are performed on an outpatient basis inside a hospital or

 clinic and involve no special preparation on your part. Head scans are a way to

 provide your healthcare provider with "photographs" of the inside of your head.
 
 
Additional Tests for Acromegaly Diagnosis
 
Still other tests, such as an electrocardiogram (ECG), chest X-ray, eye and visual

 field examination will help your doctor check your overall health. These tests 

also usually do not require hospitalization and are performed by healthcare 

specialists in different fields. These specialists and/or technicians will send 

all test results directly to your healthcare provider. Your healthcare professional

 will then provide you with your test results, as well as specific information 

regarding your current levels of GH and IGF-1. It's important to understand the 

results of all of your tests and learn your GH and IGF-1 levels so that you can

 become a more knowledgeable patient.

 KNOW YOUR TREATMENT OPTIONS

 When treating acromegaly, your healthcare professional is most concerned with

 managing the disease to preserve your overall health.

Surgery – The goal of surgical treatment for acromegaly is to remove the pituitary

 tumor, reducing the associated symptoms such as headache, and normalize GH and 

IGF-1 levels. This type of surgery is called transsphenoidal. With this type of

 procedure, the surgeon reaches the pituitary through the nose (transnasal) or

 through an incision in the upper lip (translabial) and is able to remove the

 tumor, relieving pressure and immediately reducing GH levels.

Medical Therapy – There are different medical treatments used to treat acromegaly.

 Your doctor can tell you about how each of them works.

Radiation Therapy – Radiation is most commonly used as adjuvant therapy (following

 surgery) in those cases where some tumor is still present and medications are not

 working. The full effect of this therapy may not occur for many years.
Everyone responds to treatment differently, so you and your healthcare professional

 should work together to determine which treatment option or combination of 

approaches is right for you.

ADAPTING TO LIFE WITH ACROMEGALY

Acromegaly affects not only your physical body, but it may also impact your

 self-image and emotional well-being. It's useful to acknowledge the impact that

 acromegaly has on your life.
 

This page highlights the emotional impact of living with a chronic disease, and

 suggests how you can adapt to living with acromegaly. Topics covered include:
Responding to the Diagnosis
Dealing with Setbacks
Giving Back
 
 

Responding to the Diagnosis

A diagnosis of acromegaly is a serious event. However, for many people, it may

 actually come as a relief; having lived for a long time with the symptoms, you

 finally have an explanation for what's been causing them.

   In general, people who have been diagnosed with a chronic illness respond in one

 of three ways: resignation, denial, or adaptation.

Resignation. Resigning yourself to having an illness is part of the process of

coming to terms with your diagnosis and admitting that you have to adapt your life.

 Resignation isn't the same as acceptance, as it can be somewhat dispiriting, 

especially in the beginning, but it is a start towards dealing with the situation

 and finding a sense of emotional well-being. It's normal to feel a certain amount

 of helplessness when we're confronted by difficult facts. But by acknowledging

 these feelings, and understanding that they're natural, you can begin to move

 from resignation towards true acceptance.

Denial. Some people react to illness by refusing to acknowledge that anything is

wrong. Denial is usually based on fear or anger, and these are emotions that can

 be especially difficult or feel inappropriate to express. To overcome the emotions

 behind denial, many people find that connecting with others who have acromegaly,

 or another chronic illness, can be of immense value. It's important to understand

 that these feelings are natural. By expressing them in a supportive environment,

 you gain energy, humor, and a sense of peace. Even if you don't have access to a

 support group, simply recognizing that it is normal to have unpleasant feelings 

can help make you feel better.

Adaptation. As you are able to recognize and deal with your disease and the impact

 it has on your life, you can move toward a healthier emotional state. Instead of

 "surrendering" to your disease, you can take an active role in managing it.

 Adaptation is a process, but the reward is a sense of living more fully, and the

 possibility that, by taking charge of your emotional life, you may have more

 energy to do the things that may help improve your symptoms and prognosis.