Acromegaly

Diagnosis

Diagnosis involves the steps that your healthcare professional takes to find out if you have acromegaly. Your healthcare professional asks about your health history and does a physical exam. You also may need the following tests:

• IGF-1 measurement. This blood test measures the level of IGF-1 in your blood. A high IGF-1 level can mean that the level of growth hormone also is high. This can be a clue for acromegaly.
• Growth hormone suppression test. This blood test measures your growth hormone level before and after you drink a type of sugar water called glucose. In people who don't have acromegaly, the glucose drink typically causes the growth hormone level to fall. But if you have acromegaly, your growth hormone level tends to stay high.
• Imaging tests. Magnetic resonance imaging (MRI) can help pinpoint the location and size of a tumor in your pituitary gland. If no pituitary tumors are seen, you may need more imaging tests to look for other types of tumors. Your healthcare professional also may recommend X-rays of the hands and feet. These can help check for bone growth.

Treatment

Acromegaly treatment aims to improve symptoms and treat or prevent complications. The goal is to lower growth hormone and IGF-1 back to their proper levels and keep them there.

To help lower your growth hormone (GH) and IGF-1 levels, treatment options often include:

• Surgery to remove the tumor that's causing symptoms. Most often, this is the first treatment for acromegaly that's caused by a pituitary gland tumor.
• Medicine to help lower hormone levels. This is usually an option if surgery doesn't bring down growth hormone to the right level.
• Radiation to shrink the size of the tumor. Often, this a treatment choice if surgery isn't an option. It's also an option if surgery can't remove the whole tumor or if medicine doesn't help enough.

Some people need a mix of these treatments. Your treatment plan depends on factors such as:

• The location and size of your tumor.
• How serious your symptoms are.
• Your age and overall health.

It's common for some changes in physical features to improve with treatment. For example, swelling of soft tissue often goes down. And the skin often becomes less oily and coarse. But enlarged bones don't return to the size that they used to be.

If you also have other health conditions due to acromegaly, you may need other treatments to manage them.

Surgery or other procedures

Surgeons can remove most pituitary tumors using a method called transsphenoidal surgery. A surgeon works through the nose to remove the tumor from the pituitary gland. If the tumor causing symptoms isn't located on the pituitary gland, the surgeon recommends another type of surgery to remove the tumor.

Removing the tumor often returns growth hormone to the right level, especially if the tumor is small. If the tumor was putting pressure on the tissues around the pituitary gland, removing the tumor also helps relieve headaches and vision changes.

Sometimes, surgeons can't remove the whole tumor. When this happens, the level of growth hormone may still be too high after surgery. Another surgery, medicines or radiation treatments may be needed.

Medicines

Medicine can help lower hormone levels or block the hormones' effects. Your healthcare professional may recommend one or more of the following:

• Medicines called somatostatin analogues that cause the body to make less growth hormone. In the body, a brain hormone called somatostatin limits the production of growth hormone. The medicines octreotide (Mycapssa, Sandostatin) and lanreotide (Somatuline Depot) are lab-made versions of somatostatin. Taking one of these medicines signals the pituitary gland to make less growth hormone. That also helps lower IGF-1. These medicines also might make a pituitary tumor smaller. You take the medicines by mouth or receive a monthly shot.
• Medicines called dopamine agonists that lower hormone levels. The medicines cabergoline and bromocriptine (Cycloset, Parlodel) may help lower levels of GH and IGF-1 in some people. These medicines also may help make a tumor smaller. You take dopamine agonists by mouth, and the doses often are high. That can raise the risk of side effects. Side effects can include upset stomach, vomiting, stuffy nose, tiredness, dizziness, sleep problems and mood changes.
• Medicine called a growth hormone antagonist that blocks the action of growth hormone. The medicine pegvisomant (Somavert) blocks the effect of growth hormone on the body's tissues and results in lower IGF-1 levels. Pegvisomant doesn't lower the level of growth hormone or shrink tumor size. It may affect the liver, so your healthcare professional monitors your liver health with blood tests while you're on pegvisomant. The medicine is given as a daily shot and may be used along with other medicines.

Therapies

Radiation therapy destroys any leftover tumor cells after surgery to remove the tumor. It also slowly lowers the level of growth hormone. It may take months or years for radiation to improve acromegaly symptoms in ways that you notice.

Radiation often lowers levels of other pituitary hormones too — not just growth hormone. If you get radiation, you'll likely need regular follow-up healthcare visits. These visits let your healthcare professional check your hormone levels and make sure that your pituitary gland is working right. Follow-up care may last for the rest of your life.

Types of radiation therapy include:

• Stereotactic radiosurgery. Stereotactic radiosurgery uses 3D imaging to deliver a beam of high dose of radiation to the tumor cells. This treatment also limits the amount of radiation to the healthy tissue that surrounds the tumor. Most often, stereotactic radiosurgery is given in a single dose. This type of radiation may bring growth hormone back to the right level within 5 to 10 years. The most common technique that healthcare professionals use to give this type of radiation is called Gamma Knife. It doesn't involve the use of a knife.
• Proton beam radiation. This type of radiation uses tiny particles called protons that point to the tumor. It may cause less damage to the pituitary gland and the tissue that surrounds it than does conventional radiosurgery.
• Conventional radiation therapy. This type of radiation involves receiving small doses of radiation over 4 to 6 weeks. The risk of damage to the pituitary gland is higher than it is with proton beam radiation and stereotactic radiosurgery. You may not see the full effect of conventional radiation therapy for 10 or more years after treatment.

Preparing for your appointment

You'll likely first see your primary healthcare professional. Or you may be referred right away to a doctor called an endocrinologist who finds and treats hormone conditions.

It's good to prepare for your appointment. Here's some information to help you get ready and to know what to expect from your healthcare professional.

What you can do

• Be aware of any restrictions before the appointment. When you make the appointment, ask if there's anything you need to do to prepare for tests. For instance, you may be asked to stop eating for a certain number of hours before a test. This is called fasting.
• Write down your symptoms. Keep track of anything that causes you discomfort or concern. This may include headaches, vision changes or discomfort in your hands. Write down all of your symptoms, even if they don't seem related to the reason for which you made the appointment.
• Write down key personal information, including any changes in your sex life or menstrual cycle.
• Make a list of all medicines, vitamins and supplements you're taking.
• Take along old pictures that your healthcare professional can use to compare against your appearance today. Your healthcare professional likely will be interested in photos from 10 years ago through the present.
• Take along a family member or friend if you can. This person may remember something that you miss or forget.
• Write down questions to ask your healthcare professional.

Making a list of questions helps you make the most of your time with your healthcare professional. For acromegaly, some basic questions to ask include:

• What's the most likely cause of my symptoms? Are there other possible causes?
• What tests do I need? And should I see a specialist?
• What treatments are available for this condition? Which approach do you recommend?
• How long will I need treatment before my symptoms improve? Could treatment help me look and feel as I did before I developed symptoms of acromegaly?
• Will I have long-term complications from this condition?
• I have other health conditions. How can I best manage the conditions together?
• Are there brochures or other printed material I can take with me? What websites do you recommend?

Feel free to ask any other questions you have.

What to expect from your doctor

Your healthcare professional is likely to ask you questions such as:

• What are your symptoms, and when did they start?
• Have you noticed any changes in how you look?
• How much would you say your features have changed over time? Do you have old photos I can use for comparison?
• Have you noticed changes in your sex life, or sleep? Do you have headaches or joint pain, vision changes, or more sweating than usual?
• Does anything seem to make your symptoms better or worse?
• Do your old shoes and rings still fit? If not, how much has their fit changed over time?
• Have you had a colon cancer screening test?