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Arthrogryposis Awareness Day

Monday, June 27, 2016

Every day is a good day to learn something. Designated days help that concept become a reality. Since 2005, June 30 has been recognized as Arthrogryposis Awareness Day.

Understanding arthrogryposis

When a baby does not move enough during pregnancy, arthrogryposis can develop. Arthrogryposis is a rare condition indicated in one of 3,000 to 4,000 births that affects the joints, possibly including the hands, wrists, elbows, shoulders, hips, feet and knees. Because of the lack of movement, the joints may become contracted or tightened and inflexible.

Types of arthrogryposis

The main types of arthrogryposis are:

  • Amyoplasia: The most common type, seen in approximately one in three children with arthrogryposis. It involves an overall lack of muscular development, growth and strength, and contractures of most joints, especially the hands, wrists, elbows, shoulders, hips, feet and knees. The severity of the contractures, which are frequently accompanied by muscle weakness, can vary. Amyoplasia is believed to be caused by a loss in the connection between the nerves, brain and muscles sometime during the eighth to 11th week of pregnancy.
  • Distal arthrogryposis: This type primarily affects the joints in the hands and feet. There are 19 different specific types of distal arthrogryposis, all of them with an underlying genetic cause. Because of the way the different molecular parts of the developing muscles are affected genetically, it can be difficult to make an exact distinction between the different types. There also will be an increased risk of scoliosis.
  • Syndromic arthrogryposis: This category includes approximately 300 different conditions, all of which also have lack of fetal movement during pregnancy as the underlying cause. This category includes conditions of “joint webbing or winging,” which restricts motion, such as Escobar syndrome.

Providing help

Although the severity of the condition varies in each instance, children with arthrogryposis need a lot of medical attention, including intervention by an orthopaedic surgeon as soon as possible.

Shriners Hospitals for Children is a recognized leader in the treatment of a wide range of pediatric orthopaedic conditions, including arthrogryposis. For example, Harold van Bosse, M.D., orthopaedic surgeon at Shriners Hospitals for Children — Philadelphia is on the board of directors and medical advisor to Arthrogryposis Multiplex Congenita (AMC) Support, the primary organization offering support to those affected by arthrogryposis. In addition to providing expert, innovative care and treatment, he encourages families coping with arthrogryposis to share insights and support one another.

“The peer to peer support that families gain from the AMC Support group is phenomenal. Arthrogryposis may be an uncommon condition, but with this support group available throughout the U.S. and abroad, families are brought together by the commonality of arthrogryposis,” said Dr. van Bosse.

For the past seven years, 75 to 80 percent of Dr. van Bosse’s work at the Philadelphia Shriners Hospital has been dedicated to children with arthrogryposis.

Teams of experts

While every patient at Shriners Hospitals for Children is unique and receives a personalized care plan tailored to meet their individual needs and goals, in general terms, treatment for arthrogryposis may include splints, casts, orthotics  and/or surgery, as well as an intensive program of physical and occupational therapy dedicated to helping kids achieve maximum mobility and independence.

To learn more about arthrogryposis, please read our current Leaders In Care magazine and visit amcsupport.org. To learn about obtaining care at Shriners Hospitals for Children, please visit the Refer a patient page.