Beckwith Wiedemann syndrome famously known as BWS is generally seen in children and it is an overgrowth disorder which can lead to cancer and other complex diseases. It is often observed in congenital (disorder before the birth) state and more than 300 children per year are diagnosed with this disease every year in USA. According to statistics, one in every 13,700 children is born with BHS. BWS is very common overgrowth syndrome occurring in kids and it must be treated with utmost care as there are 600 times more chances that the kids who are suffering from BWS will get cancer.
Beckwith-Wiedemann Syndrome Causes :
- Woman who has habits like smoking cigarettes and drinking alcohol are not only ruining their own health but also damaging the health of their yet to born babies.
- Children born through in vitro fertilization have more chances to get BWS when compared to normal babies. The IVF procedure is complex and even a single mistake can lead to many fold increase in chances of BWS in babies.
- Changes in the human chromosome band 11p15 is main reason BWS in children and till now researchers had observed five distinct changes in that band that can cause BWS.
- Though researches proved that genetics can cause BWS, they are unable to find the specific defect that is causing BWS. Even today it is hard to find genetic mutation that is causing BWS in 1/3rd of cases reported.
- BWS occurs equally in male or female children.
- If the biological mother and father were suffered from BWS, then there are 85% chances that their kids will also get BWS. It is important to note that 7 out of 10 children who are suffering from BWS had at least one biological parent who had suffered from BWS.
Symptoms Of Beckwith-Wiedemann Syndrome :
Usually these five primary qualities are seen in newborn kids who are suffering from BWS. They are
- Longer tongue.
- Birth weight and body structure is bigger/higher than normal new born babies.
- Blood, sugar levels decrease after birth.
- Ears look bigger than normal and one can observe more lines and deeper pits.
- Abdominal wall defects and one can observe a small bump in that area.
It is important to note that all babies that are suffering with BWS don’t posses all these symptoms and in some cases kids face problems like hearing loss, increase in size of kidneys, abnormalities in skeletal and muscle system , port wine stain, heart related problems etc. Babies born prematurely don’t show longer tongue and if they are suffering from BMS the length of the tongue increases abnormally and within one or two months anyone can observe significant difference in its length.
It is important to note that all the children who have BWS symptoms are more prone to cancer when compared to other children. It doesn’t mean that all the children who have BMS symptoms will get cancer. According to DeBaun, babies who are diagnosed with two of the five primary symptoms are suffering from BWS and Elliot proposed that babies who are diagnosed with three of five primary qualities and two secondary symptoms must be treated with BWS. The second definition was proposed in the recent years and doctors from all over the world are following this practice only.
Diagnosing BWS is a complex procedure and involves lot of tests to confirm this syndrome. Hypoglycemia screening is common in infants and after this one doctor may recommend Hyperinsulinaemia and Hyperpituitarism tests to get better idea about the condition. If he is not sure after these tests also he may finally recommend Hypoglycaemia (Hypoglycemia) and Nesidioblastosis tests and will come to final conclusion based on these reports.
Apart from the above mentioned tests, doctors may recommend X ray, blood pressure and sugar levels test, CT/MRI scan of abdomen to confirm BWS. If the new born babies are tested positive with BWS, parents need to be very cautious otherwise the situation can become worse and life threatening. In most of the cases, with little care, kids can lead a happy and joyful life.
There are so many instances where kids lost their lives because of negligence to BWS symptoms and the death may be occur due to tumors, kidney problems, macroglossia etc. This happens in the rare occasions and only less than 2% of children who are suffering from BWS come into this category. Most of the deaths are happening because of lack of knowledge about BWS in pregnant mothers.
- Abdominal scans for every six months until kids attain ten years of age.
- Continuous checking of alpha-fetoprotein levels in kids until they attain 5 years.
- If any symptoms of BWS are observed in newborn babies, hypoglycaemia test is compulsory.
In 1960’s, Dr. John Bruce Beckwith (Pathologist, American) and Dr. Hans-Rudolf Wiedemann (pediatrician, Germany) reported this syndrome independently and named it as EMG syndrome. Later the name of this syndrome changed to Beckwith Wiedemann Syndrome as a gratitude for their research.BWS doesn’t have any significant classifications/types of now.
As already mentioned, chances to get cancer is high in the kids who are suffering from BWS. Both benign and non-benign cancerscan occur in children and the chances of getting a tumor less than ten years old is 7.5%. The risk of getting cancers is reduced once they attain teenage and the chances to get cancer thereafter are similar to that of general public. Kids, who are suffering from BWS, usually get tumor in kidneys (40%, wilms tumor) and in lever (25%, Hepatoblastoma). Other important areas where the risk of cancers/problems is high are adrenal cortex (Adrenocortical carcinoma), immature nerve cells (Neuroblastoma) and muscles are attached to bones(rhabdomyosarcoma).
Home Remedies :
If proper care is taken, Kids who are suffering with BWS can also become adults without any side effects and their IQ levels and intelligence will be normal (without any effect of this syndrome).Pregnant women need to be very cautiousso that the BWS chances (and there by cancer chances)will get reduced.
Abdominal defects present in children can be cured with neonatal repair and it is better to opt for this method as early as possible. If the tongue of kids is very large, methods like breathing tubes, special nipple feeding etc are recommended. Periodic checking for BWS suffering children is necessary so that they can lead a happy life throughout their life.