Boy, 8, whose severe spinal deformity would have crushed his chest over time undergoes life-saving surgery (and now he's as tall as his classmates)

Boy, 8, whose severe spinal deformity would have crushed his chest over time undergoes life-saving surgery (and now he's as tall as his classmates)

• Dempsey Wells, from Melbourne, suffers from Distal Arthrogryposis Type 2A 
• The disease affects the bones with many sufferers having club feet and curves 
• The traditional treatment is for doctors to fix the spine in place with metal rods
• But new method used adjustable metal rods that change as Dempsey grows
  
  
  
  An eight-year-old boy has had life-saving surgery to stop him being crushed to death by his own spine.
  Dempsey Wells, from Melbourne, suffers from Distal Arthrogryposis Type 2A, or Freeman Sheldon Syndrome. 
  The disease affects the bones with many sufferers having club feet, curved or hooked joints and muscle weakness.
  
  Dempsey's spine is curved and would have eventually crushed his internal organs if left alone. 
  The traditional treatment is to fix the spine in place with metal rods - but this would have left Dempsey unable to grow taller.
  
  A new method, pioneered by a French surgeon and implemented for the first time in Australia, has given Dempsey room to grow with adjustable metal rods that change as his body does.
  'The surgeon showed me the X-rays taken in surgery, and it was absolutely amazing,' Dempsey's mother told Seven News.

  
  Since the operation three months ago, Dempsey has grown 12 inches. Asked why he was so happy, he said: 'Because I am a bit taller!' 

  Dempsey used to find physical activity very hard but has since been horse-riding, playing basketball and even AFL. 

  'We never thought he'd be doing Auskick or playing on the MCG at half time, so it was pretty phenomenal,' said his mother.

  'There were quite a few tears around.'
  

  Freeman-Sheldon syndrome

  Freeman-Sheldon syndrome is a condition that primarily affects the face, hands, and feet. People with this disorder have a distinctive facial appearance including a small mouth (microstomia) with pursed lips, giving the appearance of a 'whistling face.' 

  For this reason, the condition is sometimes called 'whistling face syndrome.'

  People with Freeman-Sheldon syndrome may also have a prominent forehead and brow ridges, a sunken appearance of the middle of the face (midface hypoplasia), a short nose, a long area between the nose and mouth (philtrum), deep folds in the skin between the nose and lips (nasolabial folds), full cheeks, and a chin dimple shaped like an 'H' or 'V'.

  Freeman-Sheldon syndrome is also characterized by joint deformities (contractures) that restrict movement. People with this disorder typically have multiple contractures in the hands and feet at birth (distal arthrogryposis). 

  These contractures lead to permanently bent fingers and toes (camptodactyly), a hand deformity in which all of the fingers are angled outward toward the fifth finger (ulnar deviation, also called 'windmill vane hand'), and inward- and downward-turning feet (clubfoot). 

  Affected individuals may also have a spine that curves to the side (scoliosis).

  
  
  
  
  
  
  
  
  
  
  
  
  The disease affects the bones with many sufferers having club feet, curved or hooked joints and muscle weakness
  
  
  
  
  
  These X-Rays show Dempsey's spine before (left) and after (right) the pioneering surgery
  
  
  
  
  Before the surgery, Dempsey had to wear this headgear to stretch his spine as much as possible
  
  

  
  

  
  
  SOURCE : DAILY MAIL , 25 JULY 2018