Prader-Willi syndrome (PWS)

Prader-Willi syndrome (PWS) is a rare, complicated condition that affects many parts of your body. It stems from a problem with one of your chromosomes (a strand of DNA that carries your genes). It can cause physical issues, like extreme hunger and weak muscles, as well as learning and behavioral problems.

Only about 1 in 10,000 to 30,000 people in the world have PWS.

Causes

PWS usually happens because of a problem with your genes. In most cases, doctors don’t know what causes it, but about 2% of people with PWS have it because they didn’t get the right copies of a certain chromosome from their parents.A head or brain injury can also cause the syndrome.

Signs and Symptoms

A baby may show signs of PWS early on. He may have almond-shaped eyes, his head may get narrow at the temples, his mouth might turn down at the corners, and he might have a thin upper lip.He also may have these symptoms:

  • Poor muscle tone or limbs that hang loosely when you hold him
  • Inability to suck or nurse well, so he gains weight slowly
  •  Eyes that cross or wander
  • Appearance of being tired all the time
  • Weak or soft cry
  • Poor response to you

In early childhood, kids with PWS start to show other signs. It affects a part of their brain called the hypothalamus — this is what tells you you’re full when you eat enough food. People with PWS don’t get the feeling of being full, so the most common signs are nonstop hunger and weight gain.child with PWS may always want more food, eat big portions, or try to hide or store food to eat later. Some kids even eat food that’s still frozen or in the garbage.

Your child may also :

  • Be short for her age
  • Have small hands or feet
  • Have extra body fat and not enough muscle mass
  • Have sex organs that may not develop
  • Have mild to moderate learning problems
  • Be slow to learn to talk, sit, stand, or walk
  • Have temper tantrums
  • Pick at her skin
  • Have sleep problems
  • Have scoliosis — a curved spine
  • Have too little thyroid or growth hormones

Because their sex organs don’t develop, women with PWS may never get a period. Or they may start their periods much later than others. Men who have PWS may not have facial hair. They may have small sex organs, and their voices may not change in puberty. People who have PWS usually aren’t able to have children.