Actor Irwin Keyes Dies from Rare Pituitary Disorder

Character actor Irwin Keyes has died at the age of 63. Keyes was perhaps best known to TV viewers for his recurring role as Hugo, the burly, but dim-witted bodyguard of George Jefferson on the CBS sitcom The Jeffersons. He also had parts in Woody Allen‘s Stardust Memories, the Coen brothers’ Intolerable Cruelty and  the  2003 cult-classic House of 1000 Corpses along with Rob Zombie.

Keyes suffered from a rare pituitary disorder called acromegaly. His niece posted this on his Facebook page:

“It’s with deep sympathy that I’m writing this post. Early this morning my family was informed that our beloved uncle, brother, teammate and friend, Irwin Keyes, has had his last curtain call. He is now an angel for us all and will be watching down. Our family wishes to thank all of you who have been a part of his life. He will be missed very much.”

What is acromegaly?

360px-Endocrine_growth_regulationAcromegaly is a hormonal disorder that results from too much growth hormone (GH) in the body.  The pituitary, a small gland in the brain, makes GH.  In acromegaly, the pituitary produces excessive amounts of GH. Usually the excess GH comes from benign, or noncancerous, tumors on the pituitary. These benign tumors are called adenomas.

Acromegaly is most often diagnosed in middle-aged adults, although symptoms can appear at any age.  If not treated, acromegaly can result in serious illness and premature death.  Acromegaly is treatable in most patients, but because of its slow and often “sneaky” onset, it often is not diagnosed early or correctly.  The most serious health consequences of acromegaly are type 2 diabetes, high blood pressure, increased risk of cardiovascular disease, and arthritis.  Patients with acromegaly are also at increased risk for colon polyps, which may develop into colon cancer if not removed.

Scientists estimate that three to four out of every million people develop acromegaly each year and about 60 out of every million people suffer from the disease at any time

What are the symptoms of acromegaly?

The name acromegaly comes from the Greek words for “extremities” and “enlargement,” reflecting one of its most common symptoms—the abnormal growth of the hands and feet.  Swelling of the hands and feet is often an early feature, with patients noticing a change in ring or shoe size, particularly shoe width.  Gradually, bone changes alter the patient’s facial features:  The brow and lower jaw protrude, the nasal bone enlarges, and the teeth space out.

Overgrowth of bone and cartilage often leads to arthritis.  When tissue thickens, it may trap nerves, causing carpal tunnel syndrome, which results in numbness and weakness of the hands.  Body organs, including the heart, may enlarge.

acromegalyOther symptoms of acromegaly include

  • joint aches
  • thick, coarse, oily skin
  • skin tags
  • enlarged lips, nose, and tongue
  • deepening of the voice due to enlarged sinuses and vocal cords
  • sleep apnea-breaks in breathing during sleep due to obstruction of the airway
  • excessive sweating and skin odor
  • fatigue and weakness
  • headaches
  • impaired vision
  • abnormalities of the menstrual cycle and sometimes breast discharge in women
  • erectile dysfunction in men
  • decreased libido

What causes acromegaly?

Acromegaly is caused by prolonged overproduction of GH by the pituitary gland.  The pituitary produces several important hormones that control body functions such as growth and development, reproduction, and metabolism.

In more than 95 percent of people with acromegaly, a benign tumor of the pituitary gland, called an adenoma, produces excess GH.  Most pituitary tumors develop spontaneously and are not genetically inherited. Pituitary tumors are labeled either micro- or macro-adenomas, depending on their size.  Most GH-secreting tumors are macro-adenomas, meaning they are larger than 1 centimeter.

Depending on their location, these larger tumors may compress surrounding brain structures.  For example, a tumor growing upward may affect the optic chiasm-where the optic nerves cross—leading to visual problems and vision loss.

Compression of the surrounding normal pituitary tissue can alter production of other hormones.  These hormonal shifts can lead to changes in menstruation and breast discharge in women and erectile dysfunction in men.  If the tumor affects the part of the pituitary that controls the thyroid—another hormone-producing gland—then thyroid hormones may decrease.  Too little thyroid hormone can cause weight gain, fatigue, and hair and skin changes.  If the tumor affects the part of the pituitary that controls the adrenal gland, the hormone cortisol may decrease.  Too little cortisol can cause weight loss, dizziness, fatigue, low blood pressure, and nausea.

How is acromegaly treated?

Currently, treatment options include surgical removal of the tumor, medical therapy, and radiation therapy of the pituitary.

Goals of treatment are to

  • reduce excess hormone production to normal levels
  • relieve the pressure that the growing pituitary tumor may be exerting on the surrounding brain areas
  • preserve normal pituitary function or treat hormone deficiencies
  • improve the symptoms of acromegaly

Source: National Institute of Diabetes, Digestive, and Kidney Diseases

Michele R. Berman, M.D. was Clinical Director of The Pediatric Center, a private practice on Capitol Hill in Washington, D.C. from 1988-2000, and was named Outstanding Washington Physician by Washingtonian Magazine in 1999. She was a medical internet pioneer having established one of the first medical practice websites in 1997. Dr. Berman also authored a monthly column for Washington Parent Magazine.

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