Beckwith Wiedemann Children's Assn of NZ (Inc)
Assisting families and individuals affected with BWS
Home      What is Beckwith Wiedemann Syndrome?
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What is BWS?
 
 Aged 7 weeks
 

Beckwith-Wiedemann Syndrome is a rare genetic disorder affecting approximately 1:15000 children. 

Principally it is an overgrowth disorder, with the overgrowth being manifested internally (as in large organs) externally, and combinations of both.

Is BWS life threatening?

The short answer is yes.  BWS does have many life threatening aspects, but with correct screening  the majority are treatable, manageable, or  even curable.  With early detection and management of Beckwith-Wiedemann Syndrome, there is every chance that a child born with BWS will grow to be a normal healthy adult. It is critical to the childs well being that the screening protocol is adhered to. Early detection of the life threatening cancers that BWS children are at risk of can be managed easily if caught in the early stages.

What is the long term prognosis?

BWS is also unusual in that it appears to be a “true” childhood syndrome.  Unlike many other syndromes, in general,  children with BWS ‘grow out of’ most of the characteristics and risk factors associated with the syndrome.

For the child born with BWS, the risk factors are generally at their highest at birth, and decrease as the child grows. In most cases by adulthood, there is nothing that distinguishes the “BWS” adult from any other non-BWS Adult.

What causes BWS?

Current research indicates that BWS is genetic in nature.  However, while there is an inherited aspect to Beckwith-Wiedemann Syndrome, BWS is also know to be sporadic (occurs randomly with no known previous family history).

One point that is important for all parents of BWS children to realize, is that nothing that you have done in the past, or during pregnancy, has ‘caused’ your child to have BWS.

BWS and In-Vitro Fertilization

Recent research has revealed that there appears to be a correlation between IVF (In Vitro Fertilization) and the occurrence of Beckwith-Wiedemann Syndrome.  The research indicates that the chance of a child being born with BWS increases markedly in children that are conceived using IVF. 

Current estimated occurrences of BWS for natural fertilization are around 1:15000, whereas the occurrence for IVF conceived children appears to be somewhere around ten times that (1:1500).

Symptoms and Characteristics of BWS

The symptoms and characteristics of Beckwith-Wiedemann Syndrome are many and varied.  It is virtually unknown for any one child to exhibit all of the characteristics, and the individual characteristics can be manifested in widely varying degrees.  Please refer to the Symptoms/Characteristics of Beckwith-Wiedemann Syndrome page for a detailed list.

What are the health risks associated with BWS?

While BWS children can face may risks and challenges, no two children are exactly alike.  Some may face many challenges, while others face very few. 

All children with Beckwith-Wiedemann Syndrome face an increased risk of developing tumors, but by following the recommended screening protocol, medical professionals are given the very best chance to discover these tumors while they are easily managed.

For more details of some of the risks and challenges that a BWS child may face please refer to the Health Risks page.