Beckwith-Wiede...what now?
Beckwith-Wiedemann Syndrome (BWS) is a rare (1 in 15,000) overgrowth disorder with several other, distinct characerteristics which Zoey has, including the overgrowth, omphalocele, enlarged tongue, stork bites on the eyes/face, hypoglycemia and a few others. BWS, fortunately, will not affect Zoey mentally as other syndromes do. It mostly affects the physical/appearance.
Zoey is big (15 lbs and 24" at only 9 weeks) and she will continue to grow rapidly. The overgrowth typically slows down around puberty. Children with BWS also typically enter puberty earlier than other children. And its not just her body that is growing so rapidly. Her organs, brain, everything grows very fast. The skull of BWS children often has ridging which is from the skull accomodating the growing brain.
Zoey also has the enlarged tongue that is typical of children with BWS. She may face a tongue reduction surgery in the near future because the large tongue can cause problems with eating and speech. More often, however, it causes problems with the way the teeth and jaw grow. Randy and I are still researching this aspect and trying to figure out what we feel will be best for Zoey...surgery or wait and see. There is a doctor in Missouri that does a lot of work with children with BWS and we may try to talk to him to get his thoughts on whether Zoey would be a good candidate for the surgery. We also found out that the WORLD headquarters for the Craniofacial Institute is here in Dallas. That means the BEST of the best of craniofacial doctors are right here in our backyard so, if Zoey needs the surgery, she will be in good hands.
The storkbites on her eyes will fade and are a non-issue. Her hypoglycemia was controlled shortly after she was born.
Her omphalocele was, of course, repaired and has healed beautifully!
The worst thing about Beckwith-Wiedemann...the scary part...is that children with BWS are at a much higher risk of developing certain types of childhood cancer. 600% greater risk than the general population. The two most common types of cancer are Wilms tumor (kidney) and hepatoblastoma (liver). Because BWS causes rapid growth, that means any tumors that develop will grow rapidly as well. Therefore, Zoey will be monitored very carefully. She will have bloodwork drawn every 6 weeks to check for signs of hepatoblastoma and she will have an abdominal and kidney ultrasound done every three months to check for tumors on her kidneys and liver. She will be checked this frequently because these cancers, if caught early, have a good chance of being cured. Once Zoey turns 4, her chance of developing hepatoblatoma decreases down to almost normal chances. After 8, her risk of developing Wilms tumor goes down to the general population level. So she will have the bloodwork drawn until she is 4 and will have ultrasounds done until she is 8. If she reaches 8 without developing cancer, she should be in the clear.
And yes, if IS very surreal to write..."if my child reaches 8 without getting cancer..."
It's the single-most frightening thing I've ever had to contemplate.
The good news is that most children with BWS do not develop cancer. Our geneticist said the risk is 1%. Other researchers say 7-10%. The aggressive screening is only to ensure that, if there is a tumor, we will catch it early.
There is more good news...or should I say "God" news because I don't think this is merely coincidence...
Dr. Vaughan, Zoey's surgeon, was NOT supposed to be her surgeon. He was only her surgeon because Dr. Black was not there when Zoey was born (If she would've been born when she was "supposed to" then Dr. Black would've been her doctor.) As it turns out, though, Dr. Vaughan is not only very knowledgeable about BWS (which is amazing because it is so rare and most doctors don't know anything about it...as a matter of fact, Zoey's pediatrician has one seen one cause of it when he was training and her neonatologist was adament that Zoey did NOT have BWS) but Dr. Vaughan has worked with children who have developed cancer as a result of BWS. He did extensive research on it during his fellowship and has published a paper on it. As a matter of fact, the timing of the screening protocol (every 6 weeks/3 months) is followed in large part BECAUSE of his research.
So, "just by chance" we ended up with a surgeon who is probably one of the most knowledgeable doctors about BWS then any other doctor in this area. How cool is that?
It reassures us that God is still very much in control. He has not abandoned Zoey. Nor do we believe that he will. He has brought her, and us, so far and I know he will continue to see us all through and sustain us when it gets to be too much to bear.
Please keep Zoey in your prayers. That she will remain healthy. Please pray for strength for Randy and I. And please pray for Sam. I know it must be hard for him to see Zoey get so much "attention".
I hate that my little girl will have to endure years of testing. I hate that we will live in fear of cancer. But I'm determined that this will NOT rule our lives. That Zoey will NOT be defined by a syndrome.
She is our beautiful little girl. A precious miracle; so full of LIFE!
And we are so very blessed to have her...
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