Gigantism and Acromegaly

Source:  Gigantism and Acromegaly    Tag:  gigantism treatment

Väinö suffered from a medical disease known as "pituitary gigantism". This disorder is caused by a benign tumor of the pituitary gland, where Human Growth Hormone (HGH) is produced. Many persons, perhaps 10-25% of the population, are found at autopsy to have small, benign pituitary tumors, or adenomas, which do not produce any hormones and which are not large enough to cause damage to adjacent structures.

However, if a pituitary adenoma arises that produces HGH, the effect will be to stimulate growth of the skeleton. If the disease occurs before puberty and before the growth plates have fused, the disease is known as "gigantism" and such person will grow to abnormally large size if left untreated. If the disease occurs after puberty, the disease is known as "acromegaly". In the latter condition, the long bones cannot grow longer, but all bones can grow thicker. Also, soft tissue will grow thicker, so persons so affected will report increased foot and hand size, and there are often facial changes, including thickening of the nose and lips, as well as growth of the bones in the jaw, cheek, and forehead.
If untreated, gigantism and acromegaly can lead to heart failure as the heart struggles to pump blood for a body growing larger than the heart was designed for. Diabetes can occur, because excessive HGH has an adverse effect on regulation of blood sugar levels.

In addition, if the adenoma grows too large, it can destroy the normal portions of the pituitary gland. If this damage occurs, the person may not be able to produce adequate amounts of thyroid hormone, cortisone, and sexual hormones such as estrogen and testosterone. Such individuals are at risk of dying from cortisone or thyroid deficiency. In addition, they may not go through puberty and develop secondary sexual characteristics such as a beard in men, or breast development in women. Persons with early loss of estrogen or testosterone are at higher risk for osteoporosis. Of course, breaking a bone is a medical catastrophe for a very large patient, since hospitals may not be equipped to treat someone so tall, and hardware to repair a fracture may have to be custom produced, if it is available at all.

An additional medical risk is blindness. The pituitary gland sits just below the optic nerve, and a growing adenoma can exert pressure on the optic nerve, leading to vision loss or even blindness. The Biblical giant Goliath is believed to have had gigantism, with loss of peripheral vision due to optic nerve damage. This would have allowed David to approach close enough to hit Goliath with his slingshot. Väino did not suffer from poor eyesight. He was able to see the Sputnik satellite unaided in 1957, and could see well enough to drive a car.

In the era before modern treatment was available, most patients afflicted with gigantism or acromegaly did not live very long lives. It was not uncommon for death to occur within 20-25 years after onset of the disease. Väinö Myllyrinne was exceptionally long lived for a person with gigantism in an era when modern treatment did not yet exist. Most people with his disease died at a much earlier age and never reached middle age, as Väinö did.

Today, treatment consists of surgery, radiation, and medication. If the adenoma is small enough, surgery can be curative in about 50% of patients. Even if the adenoma cannot be removed entirely, surgery to remove as much tumor as possible is usually attempted. Surgery is performed via the nose and does not involve opening the cranium (skull). Patients are usually released from the hospital in a few days.

Medication, particularly the drug octreotide, can reduce the secretion of HGH and a related hormone, IGF1. IGF1 is produced by tissues such as liver, in response to HGH, and elevated IGF1 is actually what makes the person grow too large. Octreotide can be given to shrink an adenoma and bring down HGH and IGF1 levels before surgery, and it can be used to control these hormones if surgery alone is not effective.

Radiation is sometimes used to destroy bits of adenoma not removed by surgery, and can also be used when an adenoma is too large to be operable. Traditional radiation needed 3-5 years to become effective, so most patients who received it also needed medication to control HGH and IGF1 during the interim period after radiation therapy. Newer forms of radiation, such as Gamma Knife, more precisely pinpoint the adenoma and can achieve control in less time, within about a year.

As a result of these treatment options, very few patients with gigantism in industrialized countries ever grow to the fantastic heights of the past. They will of course be taller than their peers and family, but hopefully the disease can be caught in time so that the person can lead a fairly normal life. Some patients, particularly teenaged boys, may be mistaken for simply being tall, so that no one suspects a problem until the person has surpassed two meters (6'7") in height.

When treatment is successful, the abnormal growth stops, and the person then has a more normal life expectancy. Of course, if problems such as low thyroid or cortisone have developed, these need to be treated on a lifetime basis, but such persons will be in better health than someone who never received any treatment at all. As a result, patients with gigantism reaching middle and even advanced age are becoming more common than in the past, when the disease gave its victims a very limited life expectancy.

In areas without advanced medical care, patients may still grown to very tall heights. Väinö lived in a country (Finland) with advance medical care, but lived in an era (born 1909) when there was no treatment for gigantism, anywhere in the world. His nephew Kalervo, who maintains this website, has not been granted full access to his uncle's medical records. What he does recall is that his uncle suffered from diabetes toward the end of his life. He also recalls that his uncle slipped on the steps outside the house and broke his leg about six months before he died, and that he never left the hospital after being admitted shortly after the accident. He further recalls that Väinö received some sort of radiation therapy in the hospital. Being a teenager at the time, Kalervo received the medical information only second hand from his father, and was not privy to what the doctors told Väinö.

Without the hospital records, a complete reconstruction of what happened is not possible, but one can build a scenario from the clues. By the early 1960s, radiation therapy was available, and it is possible that the doctors at the hospital were trying to treat the adenoma, albeit very late in the course of the disease. It is Kalervo's recollection that the fracture never healed. Osteoporosis may have played a role, as well as the difficulty of finding orthopaedic hardware large enough for Väinö. In a patient with diabetes, an unhealed fracture might carry a risk of osteomyelitis, or infection of the bone, from which some patients in those days might never recover. Finally, it is possible that he suffered from deficiency of cortisone or thyroid hormone. Failure to replace either hormone could make recovery impossible.

James Sperber M.D.
California, USA

Many thanks to James from Kalervo.