ELEVEN MONTHS OLD

Priya has only 2 1/2 weeks before she turns one...ONE!   I was thinking to have a party for her, but instead I will be quarantining her from other kiddos so we don't have to reschedule her March tongue reduction surgery (maybe we can have a super small one though).  I am not looking forward to it one bit (putting my baby girl through what seems like a barbaric procedure), but we were told she needs it, and I took her to the best of the best (Dr. Marsh in St Louis, an expert in BWS and tongue reduction).  Our craniofacial surgeon who is doing her palate surgery also recommended a tongue reduction.  Dr. Marsh said Priya is already developing an anterior cross bite (under bite) and an open bite.  If we do the surgery before the age of two, there is a 90% chance that any damage done to her jaw will correct itself. Otherwise, it will only continue to get worse--especially after her cleft palate is closed and she has less room to accommodate her large tongue.  Hopefully this procedure will also prevent any possible airway issues she might have once they close the cleft, but it's not certain since her tongue is very thick, long, AND wide.  The surgery will not affect the thickness.  The recovery from tongue reduction is a long one, 6-8 weeks.  It will take a full YEAR for her tongue to really get to where it's supposed to be though.  2-3 months after the tongue reduction, we SHOULD be able to close her palate.


A cool thing about the tongue reduction (the only cool thing) is that I get to meet a friend I met in an online support group whose daughter will be having the same surgery the SAME day as Priya!  We will all be staying at the same place while in St. Louis, so we can cry on each other's shoulders!  This family used to live in NC and moved to TN the same time we moved here from there!  Crazy!  Who would have thought we would meet for the first time in St Louis during our daughters' surgeries?

Priya had her three month ultrasound screening last week.  She thought laying there and letting someone look at her insides was going to be the death of her, but when we went to get her labs drawn, she was happy as can be playing with toys and bubbles instead of paying attention to the nurse taking a few viles of blood from her foot.  The ultrasound results came back good.  Her AFP labs came back OKAY, but her oncologist wants to repeat them in four weeks.  AFP levels start off very, very high in BWS babies after birth and should drop down to a normal range by the age of 1.  A huge increase in the AFP may be early signs for hepatoblastoma.  Priya's have been dropping consistently, but they didn't drop as much as usual since her last labs were drawn three months ago.  SO, just to make sure they are continuing on a downward trend, they are being extra cautious and doing a redraw in four weeks instead of three months.  I am pretty sure it is all fine, but I'd lie if the "what if's" don't bother me a little.

As far as milestones, Priya is still working on crawling and walking.  She has walking while someone is holding her hands down pretty well, but is still pretty wobbly on her own.  She is making sounds like "lala" and "mummm."  She loves to eat!  Her favorite food is pizza (I can't blame her).  Her first tooth popped up this week!  She also experienced her first snow and wasn't impressed at all.  Just a few days prior to our snow, we had taken her to the park on a perfect 60-something degree day.  Crazy weather.

IT'S ALL IN THE GENES

Priya has started sitting up from lying down all by herself and starting making the sign for "milk."  She is still working on staying on her hands and knees, but she doesn't seem to have much problem doing downward facing dog poses.  You'd think hands and knees would be cake!

We saw her geneticist yesterday, who was the first person to make note of hemihypertrophy of her left face.  I have questioned off and on (one eye has always been bigger than the other, but it was hard to tell for sure otherwise until her cheeks got chubbier), but it's hard to tell unless you are looking for it, and when you are LOOKING for it, you wonder how much is mommy paranoia.  Now that the doctor has noted it, I can go back and look at her pictures from 5-6 months on and see in plainly.  It is very mild and hopefully will not get worse or cause ANY problems whatsoever.  She's such a beautiful little girl that I don't think anyone will ever get passed that enough to see her face is bigger on one side than the other.  We see the geneticist for follow-up in six months.

IT'S A COLD, COLD WORLD

Well, despite our best attempts to avoid it, Priya and I caught the cold bug.  She is tolerating it well--better than I am.  We were always a little bit anxious about the thought of her getting her first cold or flu, as we didn't know how this would affect her oxygen saturation levels, but so far, she has been doing great and staying in the 90's!

Today, we met with Priya's ENT, who did a laryngescope, and we got GREAT news!  He doesn't think the thickness of Priya's tongue is going to be a problem for her airway after palate repair!  This is the first time we have got such great news!  His recommendation now is to go conservatively regarding possible tongue reduction and to take a wait-and-see approach.  We have a second opinion with Dr. Marsh and his ENT in St Louis on Tuesday.  Fingers crossed that everyone will agree!  It would be great if all we had to worry about were her cleft palate surgeries!

Priya does have thick fluid in both ears, so we will have them put tubes in her ears during her first palate surgery, so she doesn't have to go under anesthesia twice.

In the meantime, she is finally trying to get up on hands AND knees.  It's about time that she figure out hands and knees takes less energy than planking!  She continues trying to gain improved balance while standing and learning a few words in sign language.  So far "daddy" and "up" are her signs. I am really surprised she hasn't tried doing "milk" yet.  

We had a visit over the weekend from Mama, Sister (Mike's Aunt Norma), and Uncle Jerry.  This was the first time Mama and Priya got to meet each other.  Mama and Sister hung out with Priya one night so Mike and I could have a night on the town with Uncle Jerry.  We had a great time and were sad to see everyone return to FL.

PIZZA, PIZZA!

Pizza!  Priya ate pizza with me tonight!  I would break her off tiny pieces and she just kept eating!  She would look up at me impatiently for the next bite!  NO gagging or sneezing food out of her nose!  She loved it!  She never ceased to amaze me!

ONLY THE BEST

Well, it's that time we knew would come after Priya was born...time for more surgeries.  It makes my heart sick to think what is ahead for her this year, but we knew it was coming.  I am sure there will be more good than bad, though, so I won't dwell unnecessarily or worry about it more than I have to.  And, if Priya has shown us anything, it is that she is one strong and resilient little girl!  Her craniofacial surgeon here wants to start discussing a date for closure of her cleft palate.  We still are not sure if it will be one surgery or two.  Our next appointment with him is February 4th.  His concern right now is whether or not closing her palate will lead to airway obstruction due to her large tongue. So, we meet again with her ENT on January 22nd.  He will do another laryngescope to see if anything has changed since her last one.  If not, I am sure he will recommend a tongue reduction, as he has in the past.  We want a second opinion before doing any drastic procedures, so we are taking Priya to the best of the best regarding tongue reductions and Beckwith Wiedemann Syndrome, Dr. Marsh at Mercy Children's Hospital in St. Louis.  MOST likely, if it is decided that Priya does need a tongue reduction before palate closure, we will do it in St Louis.

In the meantime, we continue to monitor her oxygen saturation from time to time, and she has been breathing great!  Her solid food eating is improving all the time, and she is starting to be able to tolerate more than purees without gagging or sneezing food through her nose too much.  She impress her mommy and daddy all the time with her determination and perseverance.

PROUD MOMMA 

Priya is accomplishing new things almost daily!  The latest ones have been clapping, standing, and taking her first steps with Mommy holding her hands!  I am pretty sure that a mom is as proud of the daily small accomplishments of her child as she would be if they were winning a Nobel Prize.  

We recently received a new bottle valve/nipple combo that I ordered from overseas, and it allowed Priya to finally be able to hold her own bottle and feed herself this week!  (Thank you, Japan, for the Pigeon brand products!  I am sorry I ordered them from Thailand!)

Before, we used a Medela specialty feeder for cleft palates, which had a much longer nipple.  Because of the cleft palate, Priya does not have the negative pressure to suck by herself.  The Medela nipple allowed us to pump a little to help her.  The chamber was too long, making it awkward for her to hold up her bottle properly; plus it has to line up a certain way for the membrane inside to work, and the nipples tore every week and cost $17-22 to replace each time.  The Pigeon nipples for cleft palate (Y cut) are short like regular nipples, so there is not a leverage issue like the other.  The bottom of the nipple is soft and the top part is hard, so when Priya's tongue or gums push against the bottom, milk flows through a one-way valve and gets trapped in the nipple and milk is squeezed into her mouth for her.  It's amazing! It is so much cheaper even though I had to order from Thailand and the shipping almost doubled the cost!  This is a pic of the two nipples for comparison.  I so wish I had used Pigeon nipples earlier!

Priya turned 10 months old today.  The three of us drove to Columbia, SC, to do some exploring.  Priya loves getting out and going for walks with her mom and dad.  Here is a link to a short clip of our walk:

https://www.facebook.com/photo.php?v=10201814682383500&l=5905088739792927130

MERRY CHRISTMAS!