Acromegaly: Background information when reading The Little Giant of Aberdeen County

Acromegaly

This article relates to The Little Giant of Aberdeen County

Truly Plaice, the protagonist of The Little Giant of Aberdeen County, is referred to as a "giant" even as a child. It is not until mid-way through the book that a physician provides the name of the disease that afflicts her: Acromegaly.

Acromegaly comes from the Latin acron, for extremity, and megas, meaning large. It was originally known as "Pierre Marie Disease" after the French neurologist who first correlated the clinical and pathological findings in 1886. The disease is rare, affecting about one in every 20,000 Americans.

The underlying cause is an over-secretion of growth hormone by the pituitary gland. In 90% of acromegaly cases, this is due to a benign tumor on the pituitary gland called a pituitary adenoma.

Symptoms progress very slowly (photographs of an untreated patient over time), making the disease difficult to diagnose. Some of its effects include:

• Soft tissue swelling resulting in the enlargement of the hands, feet, nose, lips and ears.
• General thickening of the skin.
• Pronounced brow protrusion.
• Lengthening of the jaw and wide separation of the teeth as a result.
• Hoarse and husky voice.
• Increased activity of oil-producing glands.
• Over-abundance of coarse body hair.
• Enlargement of the vital organs, including the spleen, kidneys, liver and heart.

The disease is most frequently diagnosed in middle-age patients. Very rarely, acromegaly can appear in young children. Referred to as "giantism" or "gigantism," the ailment causes abnormal growth in children who typically grow to over seven feet tall by adulthood.

Acromegaly is not fatal in itself, but it can lead to serious complications if left untreated. The most frequent cause of premature death is congestive heart failure due to an enlarged heart. Diabetes is also relatively common. Other afflictions include spinal compression and pain, osteoarthritis, muscular weakness, neuropathy, and severe eye problems which can lead to permanent blindness.

The standard treatment for acromegaly is the surgical removal of the adenoma, or a partial or total removal of the pituitary gland itself. This procedure is supplemented by the use of growth hormone suppressors such as estrogen, medroxyprogesterone, chlorpromazine & Sandostatin. Without treatment, it is rare for the individual to live past 40.

Famous individuals with acromegaly include:

• Richard Kiel ("Jaws" in the James Bond movies).
• Ted Cassidy ("Lurch" in the Addams Family TV series).
• Chinese basketball player Sun Ming Ming.
• Tony Robbins (self-help guru).

Photo: Richard Kiel

Filed under Medicine, Science and Tech

This "beyond the book article" relates to The Little Giant of Aberdeen County. It originally ran in January 2009 and has been updated for the January 2010 paperback edition. Go to magazine.