Information taken from St. Louis Children’s Hospital and Michigan Development of Community Health
WHAT IS BECKWITH-WIEDEMANN SYNDROME (BWS)?
Beckwith-wiedeman syndrome (BWS) is a genetic disorder that causes overgrowth of certain body parts. There is a wide range of symptoms and severities. The characteristics and features of BWS become less apparent as the child gets older.
WHAT ARE THE CHARACTERISTICS OF BWS?
Below you will find some characteristics of BWS.
- Large body size
- Enlarged tongue
- Uneven growth of body parts
- Rapid growth
- Low blood sugar (in newborns)
- Increased risk for tumors within the first 8 years of life
- Pits or creases in the earlobes
- Large abdominal organs (i.e., kidneys, liver, adrenal glands, and pancreas)
WHAT CAUSES BWS?
BWS is genetic; however, most children diagnosed with BWS are the first in their family to have the syndrome. Rarely is the gene passed from parent to child. BWS is often caused by changes in genes located on the #11 chromosome. The genetic change is not caused by anything the mother does or does not do during her pregnancy and there is nothing a parent can do to prevent the genetic change.
HOW IS SPEECH AND LANGUAGE AFFECTED?
A child with BWS may incorrectly produce certain sounds and it may be difficult to place the tongue tip behind the upper teeth to produce the following sounds: /t, d, n, s, z, l/. Therefore, a child may make these sounds by having the body of the tongue against the bottom of the top teeth (instead of inside the mouth). A child may require tongue reduction surgery to reduce the length, width, and bulk of the tongue. Speech therapy should not focus on keeping the tongue inside of the mouth to produce certain sounds. It should be noted that children with BWS may have speech and feeding difficulties for other reasons.
HOW IS FEEDING AFFECTED?
A child with BWS may have difficulty creating a seal around a nipple due to increased tongue size. Furthermore, he / she may have restricted movement of the tongue making it difficult to chew and control the food. Increased drooling may also occur.
HOW CAN I HELP MY CHILD AT HOME?
Routine screening is very important for early detection of cancer. Children with BWS have a 5-10% chance of developing cancer in early childhood. It is also important to have routine follow-ups with your child’s physician to monitor health and to look for any other signs of cancer.
OUR APPROACH AT KEY THERAPIES:
We will develop an individualized treatment plan for each child based off of his / her current needs. A variety of strategies and techniques will be utilized to address specific goals. A home program will be taught to caregivers to assist with carry-over of skills.