Meet X-Factor Contestant Rion Paige

Meet Rion Paige.

The young lady from Jacksonville FL blew away the X-Factor judges with the appropriately named Carrie Underwood song Blown Away.

But you might notice something a little different about this 13-year-old powerhouse singer. Rion has a congenital condition called arthrogryposis multiplex congenita, which has caused permanent damage to her arms, causing her hands to be fixed in an a bent position. She is also blind in her right eye from glaucoma.

Even the sometimes cantankerous Simon Cowell was smitten with Rion, saying:

I remember the day I met Carrie Underwood and I remember predicting this girl was going to go on and do special things with her life. And I’m gonna say the same thing about you, Rion.

Here’s Rion’s audition:

What is Arthrogryposis Multiplex Congenita?

Arthrogryposis multiplex congenita (AMC) is a disorder characterized by joint deformities (contractures) that restrict movement in the hands and feet. The term “arthrogryposis” comes from the Greek words for joint (arthro-) and crooked or hooked (gryposis).

arthrogyposisThe condition is present at birth, but does not progress over time. It is  estimated 1 in 10,000 people worldwide are affected by the disorder. Most cases occur without any family history , but there a genetic cause in a small group of patients.

Affected people have limited or absent movement in large and small joints. The surrounding muscles are underdeveloped because they are not used.  The arms and legs are frequently affected, with the ankles and wrist being the most affected.

This condition typically does not cause any signs and symptoms affecting other parts of the body.

There are probably multiple causes but all seem to be related to one of two underlying mechanisms:

  1. Decreased fetal movement
  2. Hyperthermia (increased body temperature) in the mother during pregnancy

The joints are formed early in fetal life, at about 5 to 6 weeks of gestation.The limbs continue to develop as the fetus moves in the surrounding “sea” of amniotic fluid.

However, if a joint is not moved for a period of time, extra connective tissue often can grow around it, fixing it in position.

There are many conditions can cause decreased fetal movement including:

  • “Crowded” conditions- such as a structural abnormality of womb or multiple births
  • Maternal illness- caused by a virus, drug use, or other maternal conditions
  • Low amniotic fluid (oligohydramnios)
  • Neurological or muscle problems in the fetus

Hyperthermia (high fever) in the mother, especially in early pregnancy,  has also been associated  with AMC.  There are even a few cases where prolonged hot tub use has been associated with arthrogyposis.

Although there is no “cure” for AMC, patients can be helped with selective surgery to correct conditions such as clubfoot. Physical therapy, especially in infancy, can also help improve some joint movement and muscle strength. Splints and casts can support affected limbs. Occupational therapy incorporates training in fine motor skills and activities of daily life.

For more information, click here to go to the Resounding Health Casebook on the topic.

 

 

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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