If you grew up in North America in the 1980s, there’s a good chance you’re familiar with, or even watched, the wrestling television program called the World Wrestling Federation (WWF). This sports show included popular wrestlers Hulk Hogan and “Macho Man” Randy Savage. Many of these athletes were larger than life, but one redefined the term “giant.” André Rene Roussinoff, better known by his ring name André the Giant, stood 7’4” (224 cm) with a billed weight of 520lbs (236 kg). He appeared ginormous in the ring, and as a kid, I figured he was just a huge person. In fact, Andre the Giant suffered from a rare endocrine condition called Gigantism that causes abnormal growth during childhood and adolescence.
Typically, sex hormone secretion (estrogen in females and testosterone in males) noticeably increases during puberty. This increase causes the growth plates found at the ends of the long bones (epiphysis) to fuse together slowly. An endocrine disorder occurs when the child’s anterior pituitary gland secretes too many growth hormones (GH) into the bloodstream. When long-term secretion of too much GH occurs, it accelerates the growth of muscles, bones and connective tissue if left untreated or uncontrolled. The most common cause of this condition is a benign tumor of the pituitary gland called pituitary adenoma. However, it can also be caused by neurofibromatosis, McCune-Albright syndrome (MAS), and Carney complex.
Although gigantism can be hereditary, it is still not considered to be a genetic disorder. As an underwriter, I don’t see many of these cases because of its rarity. Indeed, only three people out of one million have some form of gigantism, with only one hundred known cases in the United States. However, acromegaly, the adult form of this condition, is more commonly seen in underwriting.
Why is this an important topic for life underwriters?
While pituitary disorders such as acromegaly are uncommon, life underwriters may still come across them. My goal is to share a general overview of some of these conditions.
Acromegaly occurs in approximately fifty to seventy people per million, with three out of every million developing it each year. Similar to gigantism, acromegaly occurs when the pituitary gland produces too much growth hormone, often due to a benign pituitary tumor. The tumor’s treatment can either be surgical or radiation, but hormone replacement therapy is often administered. Some symptoms associated with the disorder include enlargement of hands, feet, and organs (e.g., the liver, kidneys, spleen, and thyroid). As well, the condition may cause peripheral neuropathies, visual field defects, and headaches. Since the symptoms may develop slowly, the disorder is often unrecognized and underdiagnosed. If untreated or uncontrolled, the progression of acromegaly can result in hypertension, cardiomyopathy, osteoarthritis, diabetes, goiter, vision loss, and premature death.
Assessing risks in persons with acromegaly
When assessing risk in persons with acromegaly, whether surgically or medically treated, the focus is on the stability of the GH levels and the presence of any residuals. Testing for insulin growth factor-1 (IGF-I), X-rays for abnormal bone/organ growth, and brain MRI/CT scans are often part of the periodic evaluation for monitoring pituitary function. The risk is significant in individuals who are recently diagnosed, diagnosed under age 20, or untreated. However, after several years of demonstrated control and stability, their risk may eventually reach that of the general population.