At the base of the human brain there lies a tiny organ called the pituitary gland. About the size of a pea, this demure little gland produces and secretes a cocktail of hormones into the bloodstream from its bony nook inside the skull, helping the body to govern many internal systems. The pituitary is perhaps most well-known for its production of the
amino acid protein somatotropin, a growth hormone which stimulates cell reproduction and bodily development.
This humble chemical factory typically produces some amount of growth hormone throughout an individual’s life, though the volume usually drops off precipitously after adolescence. In spite of its small size, however, misbehaving pituitaries have been known to cause massive consequences for their owners.
One of the more common defects is a benign tumor called an adenoma. This swelling originates in the pituitary, often producing excessive amounts of one or more of the gland’s chemical messengers.
When such a tumor spills excessive growth hormone into the body, a condition called acromegaly arises.
The effects of acromegaly vary depending on the development stage of the stricken individual. If the condition arises after the end of natural growth, the victim’s hands, feet, jaw, forehead, and ribcage will often grow outwards. But when the tumor arises earlier, in someone who hasn’t yet reached full height, a phenomenon called gigantism occurs. This state causes continuous growth, ultimately leading to an abnormally tall stature in adulthood. The extremities also grow more than the rest of the body, leading to disproportionately large hands and feet, and a characteristic large-boned face.
The extreme height and distinctive features of gigantism have been irresistible to Hollywood. Actors Richard Kiel (“Jaws” in James Bond movies) and Matthew McGrory (“Karl the Giant” in Big Fish) both had acromegaly. Even those acromegalics in other professions frequently seem to end up on the big or little screens. André the Giant and Paul Wright, both professional wrestlers, and Gheorghe Muresan, a basketball player, have all had parts in more than one movie or TV show. Carel Struycken, who played Lurch in the Addams Family movies, was literally pulled off the street when a woman abandoned her car to run after him and offer him a part in Sergeant Pepper’s Lonely Hearts Club Band.
Unfortunately fame and fortune don’t offer much protection against the life-threatening effects of acromegaly. Over time it leads to a host of systemic problems including arthritis, diabetes, and heart disease. The tumor itself, should it grow too large, can press against the optic nerves and cause blindness. Untreated acromegalics rarely live past middle-age. Early treatment could avoid most of these problems, but early diagnosis, let alone treatment, is hard to come by. Most people go for years— as much as a decade or more— between the onset of symptoms and a diagnosis.
Even someone as drastically affected as André the Giant didn’t receive a diagnosis of acromegaly until well into his pro-wrestling career, and after much damage had already been done. His death in 1993 was directly attributable to his disease.
While an adenoma can result in a surplus of growth hormone, there are some conditions which can result in just the opposite. Certain brain diseases, tumors, and trauma can cause the pituitary gland to reduce its output, which causes a host of ill effects. Most cases of growth hormone deficiency appear in adults, causing reduced bone and muscle mass, increased body fat, and some impaired brain functions. However, when this condition occurs in a child, the shortage of growth hormone usually leads to pituitary dwarfism, resulting in an abnormally short stature with normal body proportions.
Perhaps the most dramatic demonstration of the pituitary’s power occurred in a man named Adam Rainer. Born in Austria in 1899, Adam experienced abbreviated growth during his childhood, and it soon became apparent that he was destined to be a very short man. At age twenty-one he stood only 3′ 10.5″ tall.
One can only imagine that by that age Adam had come to terms with his fate, accepting that he would live out his life as a dwarf. But he was mistaken.
In his early twenties, little Adam Rainer began to grow at an astonishing rate. It seems that his malfunctioning pituitary had gone from producing too little growth hormone to producing far too much, and over the next eleven years he grew an average of 3.6 inches per year. By the time he was thirty-two Adam Rainer stood at just under 7′ 2″— a giant by some estimations— and it was at about that time that he lost the ability to stand on his own. The strain of such massive growth left him bed-ridden, and though his rate of growth did decrease, it did not stop. By the time Adam died at age fifty-one, he had gained another six inches, leaving him at 7′ 8″ tall. He was the only man in history to be classified as both a dwarf and a giant.
Today, treatment for pituitary problems is readily available, though the gradual onset of symptoms frequently leads to late diagnosis, which in turn decreases the effectiveness of treatment.
Surgical measures for gigantism have been available since Dr. Julius von Hochenegg successfully pioneered a method in 1908 where he accessed the pituitary by punching through the back of the nose, an approach still favored by surgeons today. A handful of drugs can also be used to alleviate the problems of excessive growth hormone and to try to shrink the tumor, though their track record is not wonderful. Pituitary dwarfism can be successfully treated by simply administering synthetic growth hormones, assuming it is diagnosed before skeletal development is complete.
The most astonishing thing of all may be that this entire set of dramatic symptoms is the result of a deficit or surplus of one single hormone. The pea-sized pituitary produces half a dozen distinct hormones, each of which triggers a unique set of problems should the pituitary be compromised. Acromegaly and dwarfism are but two of a plethora of potential problems. Sometimes small things can be very important indeed.