If a person experiences a sudden growth of his or her extremities or protruding body parts, the suspicion of acromegaly is justified. This is a growth hormone disorder, which is also known as Pierre-Marie syndrome, among other names. At the first signs, the affected person should immediately consult a specialist, because especially the growth of internal organs associated with the disease can have serious consequences for the patient.
What is acromegaly?
The main cause of the disease is an excess of growth hormones in the blood, which originate from the pituitary gland, the so-called pituitary gland, and enable tissue growth. Acromegaly is a special growth hormone disorder that usually appears in affected individuals around the age of 50. It is currently assumed that there are about 50 affected persons among one million people in Germany. The main cause of the disease is an excess of growth hormones in the blood, which originate from the pituitary gland and enable tissue formation. These growth hormones include human growth hormone (HGH), growth hormone (GH), somatotropic hormone (STH) and somatotropin. Often a period of up to ten years is necessary to finally diagnose the disease. The reason for this is the fact that the external signs of the disease do not become visible until late.
Causes
Acromegaly is caused by an adenoma of the pituitary gland in the majority of cases. Thus, an adenoma, which is a benign hormone-producing tumor, is the basis of acromegaly in over 95%. The formation of hormones in the adenomas cannot be interrupted by a normal control circuit, so that in the long term an overproduction of growth hormones takes place. If the present adenoma is smaller than one centimeter, it is called a microadenoma. If they measure more than one centimeter, they are again referred to as macroadenomas. In rarer cases, ectopic production of the so-called somatotropin may also underlie acromegaly. This outward production can occur via hormone-forming tumors in humans. A malignant pituitary tumor is also a possible cause. However, these malignant tumors are very rare as a cause of acromegaly.
Symptoms, complaints, and signs
In acromegaly, symptoms appear over the course of years. Initially, affected individuals experience undefinable headaches as well as increasing fatigue and exhaustion. As the disease progresses, bone pain is added. The hands and feet slowly increase in size, causing complaints such as thickening of the skin, an excess of articular cartilage and joint disease. Accompanying this is increased hair growth and often high blood pressure. Typically, the organs also grow – a so-called visceromegaly occurs, which, depending on which organ is affected, can result in complaints such as digestive disorders, jaundice or cardiac arrhythmias. In men, acromegaly is also manifested by erection problems. In women, sexual dysfunction and absence of menstruation occur. The overgrowth can be detected early on by numbness, tingling and increased sweating. On the face, changes occur in the ears, eye sockets and chin. The tongue, jaw, teeth and lips may also be affected. The multiform malformations cause various symptoms and complaints that can only be clearly attributed to acromegaly in the course of the disease. The most obvious symptom is the growing limbs.
Diagnosis and course
The characteristic symptoms of acromegaly become apparent only over a long period of time, so the diagnosis of this disease is considered difficult. During the course of acromegaly, the hands and feet of affected individuals grow steadily. In addition, changes in the face are characteristic, which include large ears, bulging orbital rims and a prominent chin. Affected individuals also often have a large tongue and a large jaw, which can alter the position of the teeth. The increasing growth is often associated with joint pain, headaches, visual disturbances or chewing disorders.Most problematic, however, is the enlargement of the internal organs, which can lead to leaky heart valves or cardiac arrhythmias. If there are first indications of a possible growth hormone excess, a specialist should be consulted immediately. The first step is to measure the amount of GH and the messenger substance insulin-like growth factor 1 (IGF-I) in the blood. If this reveals an elevated hormone level, a glucose tolerance test is performed in the further course of diagnosis. The GH level in the patient’s blood is then determined. If the GH level indicates an uncontrolled GH formation, an MRI is performed in the next step. This can diagnose a possible pituitary adenoma as the cause of the disease at an early stage.
When should you see a doctor?
In acromegaly diagnosed early, regular visits to the doctor are necessary due to the many side effects and symptoms of the disease. In addition, unusual growth in size occurs here and is soon noticeable. Visits to internists, orthodontists or other physicians are the order of the day. However, if acromegaly occurs before or after puberty, the increased size growth of the extremities and other outstanding features of the body must first be noted. This is usually not done promptly due to the rarity of the condition and the gradual onset of the disease. Trained endocrinologists are most likely to determine the cause of the growth in size of certain parts of the body. The patient usually goes to the doctor only after noticing the increased growth of certain body parts. Whether an adenoma or hormonal overproduction is present can also be determined by a physician based on the other symptoms and typical accompanying symptoms. However, since acromegaly progresses insidiously, incorrect diagnoses are often made. Individual symptoms are often not related to each other. Without hormone analysis, the doctors who are consulted usually only discover acromegaly at a late stage. If an adenoma is present, surgical, drug or radiotherapeutic treatment can be initiated. For this, an oncologist or internist should be consulted to verify the presence of tumor-related acromegaly and make an appropriate recommendation.
Treatment and therapy
There are different ways to treat acromegaly. Surgical removal of the pituitary adenoma is a common option. These operations, which usually involve access through the nose, are particularly successful for microadenomas. In addition to surgical procedures, however, there is also the possibility of treating acromegaly with medication. These include somatostatin analogues, which significantly inhibit growth hormone release from the pituitary gland. They can also bring about a reduction in adenomas, but at the same time carry the risk of gallstones. The drugs are often combined with so-called dopamine agonists, which are also used, among other things, to treat Parkinson’s disease. When dopamine agonists are used, however, liver values should always be checked for a possible increase. If treatment with somatostatin or dopamine agonists does not show sufficient success, the so-called GH receptor antagonists can also be used. These prevent growth hormones from spreading effectively in the body. If this measure also fails to bring about success, an attempt is made to remove the adenoma via high-precision radiation therapy. If hypopituitarism occurs after this radiation therapy or other surgery, hormone replacement therapy is requested. The success of all therapies must be verified by a physician by always checking the measurements of growth hormones in the blood.
Outlook and prognosis
Acromegaly causes a growth disorder in the patient. This disorder can equally affect the internal organs, leading to various complaints and complications. As a rule, this can cause the organs to shift, thereby pinching other organs. Acromegaly can therefore lead to life-threatening situations for the patient, which must be treated by a doctor in any case. In most cases, the patient’s ability to cope with stress decreases extremely and the affected person suffers from severe headaches and fatigue. The bones and joints also hurt and extremely restrict the patient’s everyday life.Furthermore, there is a giant growth, which is not infrequently accompanied by hair loss and high blood pressure. Due to the external symptoms, children are often teased. Numbness or paralysis may also occur over the entire body. Acromegaly is usually treated by surgery. This does not cause any further symptoms. However, radiation therapy is usually necessary after surgery to completely combat the disease.
Prevention
A preventive measure against the occurrence of acromegaly is not yet known. Affected persons can only make sure to consult a specialist immediately at the first symptoms. In this way, no important treatment time is wasted.
Aftercare
After successful treatment of acromegaly, steady follow-up care is recommended. This includes follow-up examinations at regular intervals. During these, the success of treatment is checked. This is the only way to take timely action if the patient’s values change. The follow-up appointments are coordinated with the responsible physician. During these examinations, blood values and the tumor of the pituitary gland are checked. Potential side effects of the medications taken are also checked. In addition, the patient’s general state of health is always examined. In this process, all relevant additional information helps the attending physician. This includes physical and mental well-being and whether other medications are being taken. Other follow-up examinations may include visual acuity checks, joint and thyroid examinations, and cancer screening. These examinations take place depending on the patient’s symptoms. In individual cases, this is decided by the attending physician and additional treatment is ordered. In addition to regular follow-up examinations with a physician, a healthy lifestyle is also recommended. This is achieved with a wholesome and healthy diet, sufficient exercise and valuable social contacts. Family and friends in particular can help patients cope with their disease. In addition, a psychotherapist can be consulted if needed or if psychological problems arise from the disease.
Here’s what you can do yourself
In order to control the course of acromegaly, the patient himself can contribute a few things. For example, participation in a support group is considered a helpful measure. In order for the treatment of acromegaly to take a favorable course, it is important for the patient to attend regular follow-up and control examinations. If necessary, an early adjustment of the therapy to possible changes can be made in this way. The patient also has the opportunity to become active himself by creating a special diary in which he enters his complaints as well as the effects of the medication taken or the results of the control examinations. Thus, the patient’s diary has a positive part in monitoring the course of the disease. The psyche of the affected person also plays an important role in acromegaly. Thus, the disease affects not only the physical, but also the psychological and emotional balance. If acromegaly has negative consequences for the psyche, professional counseling by a psychologist or doctor can be helpful. Participation in a self-help group, such as the network “Hypophysis- und Nebennierenerkrankungen e.V.” (Pituitary and Adrenal Diseases) is also considered useful. Here, the affected person makes contact with other people who also suffer from acromegaly in order to exchange ideas with them. Also recommended are a healthy diet, exercise, and support from relatives or friends to draw strength to deal with the serious disease.
