Acromegaly Treatment- Endocrinology, Neurosurgery
Acromegaly is a hormone disorder typically diagnosed in adults that occurs when your pituitary gland overproduces growth hormone. Excessive growth hormone leads to the enlargement of bones. In children, this condition is called gigantism, which causes them to outgrow their peers in height. However, as an adult with acromegaly, your height does not change. Instead, it increases the size of the bones of your face, hands, and feet.
If left untreated, high levels of growth hormone can affect other body parts, in addition to your bones. As a result, it can cause serious, even life-threatening, health issues. However, treatment can significantly improve your symptoms, including the growth of your features, and lower your chance of complications.
What Causes Acromegaly?
Acromegaly develops when the pituitary gland consistently produces excessive growth hormone (GH). At the bottom of your brain and behind the bridge of your nose lies a little gland called the pituitary gland. It makes GH, along with several other hormones, required for normal body functioning. Therefore, GH is crucial in controlling your physical development.
IGF-1, also known as insulin-like growth factor-I or IGF-I, is a hormone your liver produces in response to the pituitary gland's release of GH into your bloodstream. Your bones and other tissues grow as a result of IGF-1. Unfortunately, too much GH leads to excessive IGF-1 production, which can result in the signs, symptoms, and complications of acromegaly.
The most frequent cause resulting in excessive GH production in adults is a tumor, which can be of the following two types:
A benign or noncancerous tumor of the pituitary gland (which is medically called pituitary adenoma) is the primary cause of acromegaly. Most of the signs and symptoms of acromegaly are brought on by excessive growth hormone production due to the tumor. In addition, the tumor pressing on adjacent brain regions causes acromegaly symptoms, including headaches and vision problems.
Acromegaly is occasionally brought on by malignancies in other organs like the pancreas or the lungs. These tumors sometimes release GH. Other times, the pituitary gland receives a signal from the tumors to increase GH production by releasing a hormone called growth hormone-releasing hormone (GH-RH).
The acromegaly symptoms differ depending on how long the patient has had the condition. The most typical signs and symptoms of acromegaly are listed below. However, each person may have different symptoms.
Some signs and symptoms of acromegaly could be:
- Swelling of the hands and feet
- An increase in body odor and sweat
- Body hair gets coarser as the skin gets thicker and/or darker
- Facial features become coarser as the bones increase in size
- Protruding jaw
- Deepening of the voice
- Enlargement of the heart and other organs
- Enlarged nose, lips, and tongue
- Thickened ribs (creating a barrel chest)
- Painful joints and degenerative arthritis
- Weakness in the arms and legs and unusual sensations (carpal tunnel syndrome)
- Weakness and exhaustion
- Loss of vision
- Irregular menstrual cycles in women
- Production of breast milk in women
- High blood pressure
The subtlety of the symptoms makes acromegaly frequently undiagnosed until years later. To diagnose acromegaly, the following tests may be done in addition to a thorough medical history and physical examination:
You must undergo a blood test to determine your growth hormone levels if your doctor suspects acromegaly. You might be asked to consume a sugary solution before a series of blood samples are taken to ensure that the blood test provides an accurate result.
Drinking the mixture should prevent growth hormone release in those without acromegaly. Growth hormone levels in the blood will remain high in acromegaly patients. This is known as a glucose tolerance test.
Additionally, your physician will check the level of insulin-like growth factor 1 (IGF-1). IGF-1 levels are a relatively reliable indicator of whether you may have acromegaly.
You might undergo a brain MRI if your blood tests reveal high levels of growth hormone and IGF-1. This will show the location and size of the adenoma in your pituitary gland. A CT scan can be done instead of an MRI if you cannot have one, but it is less precise.
Your healthcare practitioner may also request the following tests:
- Sleep studies that test for sleep apnea
- Echocardiography to examine the heart
- X-rays or a DEXA (DXA) scan to examine the state of the bones
- A colonoscopy to evaluate your colon health
Treatment of acromegaly is based on your symptoms. Usually, the aim is to
- Improve your symptoms
- Bring growth hormone production back to normal
- Alleviate the pressure a tumor may be putting on nearby tissues
- Treat any hormone deficiencies
The majority of acromegaly patients get surgery to remove a pituitary tumor. Sometimes, medication or radiotherapy is required in place of or after surgery.
Acromegaly can typically be cured with surgery. However, occasionally the tumor is too big to be wholly removed, necessitating a second operation, more medical care, or radiotherapy.
Under general anesthesia, the surgeon will make a small incision behind your upper lip or inside your nose to access the pituitary gland. Your doctor will then insert an endoscope—a long, thin, and flexible tube attached with a light and camera at one end—into the opening to view the tumor. Next, the same hole is utilized to insert surgical tools and remove the tumor.
Your growth hormone levels should fall right away once the tumor is removed, and the pressure on the surrounding tissue should also decrease. Within a few days, swelling typically begins to subside, and facial characteristics frequently start to return to normal.
Surgery carries the risk of:
- Injuring the healthy portions of your pituitary gland
- Meningitis (inflammation of the sheath surrounding the brain), although this is rare
- Leakage of the fluid that protects and surrounds your brain.
Your surgeon will go over these risks and address any queries you may have.
Medication may be prescribed if your growth hormone levels are still elevated after surgery or if surgery is not an option. There are three different kinds of medicine used for the treatment of acromegaly:
1. Drugs that lower the synthesis of growth hormone (somatostatin analogs)
Somatostatin, a brain hormone, suppresses the body's ability to produce GH. Synthetic (man-made) somatostatin is available as the medications octreotide (Sandostatin) and lanreotide (Somatuline Depot). These medications prompt the pituitary gland to make less growth hormone and might even shrink a pituitary tumor. Usually, a medical expert will inject these medications once a month into the gluteal muscles in your buttocks.
2. Drugs to stop the effects of GH (growth hormone antagonist)
Pegvisomant (Somavert) prevents GH from having an impact on bodily tissues. For those who have not succeeded with conventional treatments, pegvisomant may be very beneficial. This drug, which is administered as a daily injection, does not affect GH levels or tumor size, but it can help lower IGF-1 levels and relieve symptoms.
3. Drugs that lower hormones (dopamine agonists)
Some people may benefit from a reduction in GH and IGF-1 levels after taking oral drugs such as cabergoline and bromocriptine (Parlodel). These medicines might also aid in reducing tumor size. However, these drugs typically need to be taken in large quantities to treat acromegaly, which raises the possibility of side effects. In addition, these medications frequently cause vomiting, nausea, stuffy nose, fatigue, dizziness, sleep issues, and mood swings.
You might be given the option of radiotherapy if surgery is not an option, the tumor cannot be removed entirely, or other medical treatments have failed.
Your growth hormone levels may gradually decrease due to this, but it can take several years before you see a difference, and you might need to take the medication in the meantime.
There are two primary forms of radiotherapy for the treatment of acromegaly:
This involves accurately targeting your adenoma with a high-dose radiation beam. In addition, you will need to wear a plastic mask or rigid head frame to keep your head motionless during treatment. Treatment is often completed in a single session.
This uses a similar radiation beam to the one used in stereotactic radiotherapy. However, it is wider and less precise, which increases the risk of damaging the surrounding pituitary gland and brain tissue.
As a result, it is administered in small doses over 4 to 6 weeks. This will give your tissues time to heal between treatments.
Adenomas are more frequently treated with stereotactic radiation because it reduces the possibility of damaging neighboring healthy tissue.
There are several potential adverse effects of radiotherapy. First, you will typically require hormone replacement drugs for the rest of your life because they will frequently cause a progressive reduction in the amounts of other hormones generated by your pituitary gland. Your fertility may be affected as well.
Acromegaly symptoms can frequently be reduced, and the overproduction of growth hormones can be stopped with treatment. However, you will need lifelong follow-up visits with your specialist following treatment. These will be used to check your hormone replacement therapy compliance, assess how well your pituitary gland is functioning, and ensure the condition does not recur.
Untreated acromegaly can cause serious health issues. Possible complications include:
- Elevated blood pressure (hypertension)
- Cardiac problems, specifically heart enlargement (cardiomyopathy)
- Type 2 diabetes
- High blood cholesterol levels
- The thyroid gland (goiter) increases in size
- Sleep apnea
- Carpal tunnel syndrome
- Polyps on the lining of the colon
- Increased risk of cancers
- Fractures or compression of the spinal cord
- Eyesight alterations or complete vision loss
The severity of the condition and the effectiveness of treatments to manage the symptoms determine the prognosis (outlook) for acromegaly. After receiving treatment, many patients notice a dramatic improvement in their symptoms.
When left untreated, acromegaly can substantially alter your appearance and the structure of your bones. In addition, these symptoms may significantly impact your self-esteem and quality of life. Some individuals find support networks helpful in overcoming the obstacles that acromegaly presents.
Acromegaly-related health issues like heart disease and Type 2 diabetes can negatively impact your quality of life and potentially reduce your lifespan. As a result, it is crucial to get in touch with your doctor if you notice any symptoms and to follow your treatment plan if you have already been given a diagnosis.
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