Acromegaly - This Rare Disease Can Cause Giantism and Facial Distortion

“Acromegaly?” “Yes, it’s a very rare disease, glandular in origin, activated by a defective pituitary.” “Doctor can’t you tell me in plain English what’s wrong with me and how serious it is?” “I’m trying to tell you. You have a glandular disorder, a disorder of the pituitary gland; a disease so rare that medical science knows little about it as yet.” “Is it fatal?” “Fatal, well not necessarily. It’s a progressive disease, enlarges the extremities. It’s accompanied by an increasing amount of energy, to a fabulous and dangerous degree.” “Is it curable...?”

If you are an old horror movie buff, you might recognize these lines from the 1944 film, The Monster Maker, starring J. Carroll Naish and Ralph Morgan. In the above dialogue, Ralph Morgan’s character, Anthony Lawrence, has just been told by his doctor, Dr. Adams, that he has acromegaly. Dr. Adams proceeds to try to explain this rare disease and its consequences to his patient.

In actuality, the definition recited above by Dr. Adams to his patient Anthony Lawrence is an accurate, if simplistic, description of acromegaly. While knowledge about this rare disease – rare diseases are diseases that affect fewer than 200,000 Americans – has increased since 1944, it remains a relatively obscure disorder; most patients do not receive an accurate diagnosis until the disease has progressed for several years. Indeed, acromegaly (once known as Marie Disease) is an insidious disorder caused by the over secretion of the growth hormone (GH) which is produced by the pituitary gland. The disease is characterized by abnormal enlargement of the extremities and head, particularly the facial bones and jaws; there is also thickening of soft tissues, including heart tissue.

The most common cause of hypersecretion of the growth hormone is a pituitary adenoma – a benign tumor - which affects the somatotrophic (another name for growth hormone) cells in the anterior lobe of the gland.