Talking Tongue

87 Days Old 

Gracie had her BWS initial checkup with Dr. Kalish and team. Dr. Kalish is the leading geneticist in children with Beckwith Weidemann Syndrome. She is the director of the Beckwith Weidemann Program of Excellence and has a laboratory and research team in her name here at CHOP. Most places have heard of, but hardly (if ever) seen children with BWS, while she has made it her life's work. 

We are so lucky to be here. 

They took measurements of her limbs, since BWS can cause an overgrowth of limbs. This mostly occurs in one limb over the other, causing a discrepancy in symmetry, which is called hemihypertrophy. As of this moment, she is pretty symmetrical, but the doctors will repeat the measurements when she gets a bit bigger. 

ENT also came by to see her and did a bedside scope. At this time they do not see anything concerning with her upper airway, aside from her tongue. This is great news and the hope is that once she heals from her surgery, she will no longer obstruct her own airway. She will have to have another sleep study post op to see. 

Let's talk tongue...

Gracie has macroglossia, which means she has an enlarged tongue. It is the staple physical characteristic of Beckwith Weidemann Syndrome. 

Gracie's tongue is so enlarged that it often sticks out of her mouth and when she is sleeping, or her head is in certain positions, causes her to obstruct her own airway. This stops her breathing and then causes her heart rate to drop. 

Her tongue is also the reason as to why she is at a high risk of aspirating while (PO) feeding. 

Gracie needs a tongue reduction surgery. 

It is not an easy surgery recovery wise, and is often not done this early in life, unless it affects the babies airway and feeding (it affects both for Gracie). Her surgeon is one of the best in the country and is the leader in tongue reconstruction on babies with BWS. 

Dr. Taylor will be completing her reduction. He works closely with Dr. Kalish and team since macroglossia is a staple feature of BWS, and many are good candidates for tongue reduction surgery. Dr. Taylor is also top in his field. He is the chief of the division of plastic, reconstructive and oral surgery as well as the co-director of craniofacial program at CHOP. He and Dr. Kalish are the reasons we are here. 

                                               What can we expect recovery wise? Honestly, we aren't really sure. 

She could be intubated anywhere from 5-10 days. Post op there is expected to be a lot of swelling and a lot of discomfort for Gracie. They will not keep her sedated to recover, but will try to manage her pain as best as they can. There is a fine line between pain management and over medicating, especially in an infant. I would be lying if I said that it wasn't causing me a lot of anxiety as surgery day is getting closer. 

We know this is the best choice for her and that she absolutely needs this surgery. It will undoubtably be harder for us to watch than for her.

For now, we are going to snuggle her, let her hang in her mama roo, and give her all the lovin before surgery. While she is intubated we won't be able to hold her and I'm sure comforting her will be difficult. 

Please keep Gracie Girl in your thoughts and prayers. Right now, surgery is scheduled for Wednesday morning.