St. Louis Dr. Visit Update

Eric and I traveled to St. Louis with Julia to meet with Dr. Marsh, a pediatric plastic surgeon.  One of her symptoms of BWS is having a large tongue and Dr. Marsh is pretty much one of the best people in the world who does tongue reduction surgeries.  He told us that to count, he has done about 330 tongue reductions, including 2 last week.  He has pretty much perfected a method to do it and comes highly recommended in all recommendations that I have read.  One of the strongest, which I think I mentioned in my last post, is from Dr. Beckwith, who stated that if it was his grandchild, he would listen to and do whatever Dr. Marsh recommended regarding the child's tongue.  He recommends doing it prior to 1 year for a variety of reason.

On to the day of the appointment, we left Monday (November 12)  morning a little after 8:00 am and got to St. Louis a little after 11:00.  We went and had a quick lunch and then headed to the hospital to meet with Dr. Marsh and his team.   Our appointment started at 1:00.  We met with Kristen, who is the BWS coordinator for Dr. Marsh.  She took a brief medical history on Julia and asked lots of questions.  Next, Dr. Marsh and his nurse/nurse practitioner (DeAnn) came in for the consult.  Dr. Marsh talked to us about a few different things, said he agreed with our geneticist's clinical diagnosis of BWS for Julia, and then took a look at Julia's mouth and tongue.  She cooperated very well for this, it wasn't long, but typically I don't think babies like fingers and tongue depressors being shoved in their mouth.  He told us that he does feel Julia is a good candidate for a tongue reduction surgery.  He said on his scale of 1-10, 1 being the smallest tongue he would recommend surgery on and 10 being a tongue that couldn't physically fit in the mouth, he would rate her at a 4.  He said there are 3 main reasons to do surgery and he said she fits 2 of the 3.  One is problems breathing, which thankfully doesn't affect her.  The second is jaw placement, he said right now, her top and bottom gum meet exactly.  Over time, they may stay like this, but more than likely, the lower jaw will come out further than the upper jaw, which would create a need for jaw surgery and extensive orthodontics in the future. The third is social and emotional reasons, he said she would learn to consciously think about keeping her tongue in her mouth and be able to, but when concentrating or being tired, it would more than likely come out and kids would probably make fun of her.  He said recently they have had a few 12-14 year olds come in who found out about the tongue reduction surgery on their own and requested to have it done so that they didn't have to deal with it anymore.

He explained about the surgery and what he would do.  He will basically cut a wedge shape from the front of the tongue, then trim each side of her tongue.  After all of the cutting, he will stitch the front together (where he cut the wedge from) to create a tip and he will stitch each side back together (where he trimmed).  He said she should still have normal taste buds.  He said that of kids who have tongues like this, 90% will need speech, he said if they have the tongue reduction surgery, that 50% will need speech and 50% will not need speech.

DeAnn went through the recovery process with us.  She said the first day looks pretty rough and is pretty rough, but it gets better quickly.  She said by day 2, pretty much all kids are smiling.  She said they have a playroom so they can get out of their hospital room.  They start trying to feed them as soon as they will tolerate feedings.  Feeding is still one of the logistics we need to figure out, she said often times infants who breastfeed have a more difficult recovery (more difficult in an emotional sense than a healing sense).  However, she said of the 330 surgeries done, only about 20 have been breastfed going into surgery and that of those 20, 10 went back to breastfeeding.  Eric and I are still discussing what we feel will be the best way to handle this.  It is hard for me to think of weaning her completely prior to the surgery since it took me staying in the hospital with her for 4 days so that she could breastfeed to get her to come home.  Julia will probably be in the hospital in St. Louis for 2-3 days.  The NP recommends that one of us stay with her 24 hours a day.  There is a Ronald McDonald house that we most likely will also be able to stay in during this time and doing a little bit of sleeping and rotating visiting her.  We can bring the boys to the RM house, but would really need to bring someone just to take care of them so we haven't decided what we will do with the boys during this time yet.  The stitches he uses typically take about 6 weeks to fully dissolve so until that time, her tongue would look a little rough- the pictures they showed us, the stitches look like little white lines all over her tongue.  The tongue will be functioning normally during this time, but can take up to a year to reach the final shape that it will be.

Obviously, this is a big surgery and we aren't making this decision lightly, but we plan on taking Julia back to Dr. Marsh in the spring, probably March or April, to have this surgery done.  It is scary to think of putting her through surgery and unfortunately, the recovery for this surgery will be much rougher than her previous surgery, but long term, Eric and I both feel this is the best decision for her.  We are also very grateful to be so close to St. Louis because people fly from all over the world to have Dr. Marsh perform this surgery on their child.

Our next step is to actually schedule the surgery. We are trying to figure out the best date to do it, but are leaning toward late March to early April, mostly based on Eric's school schedule right now.  One really nice thing about his office and they are very easy to contact and get answers to questions.  We already have office phone numbers, cell phone numbers, and email addresses to contact them.  They also really took their time with us which we both appreciated it.  We were in their office for 3 hours and 45 minutes.  I'm sure we will be asking lots of questions to get ourselves as prepared as we can be in the next few months.

A bit of an update of her 3 appointments last week:
Speech- the SLP said that she is doing well.  She is functionally eating, her form isn't the greatest, but she is able to do it.  We are switching to a different style of bottle to hopefully help correct her form.

Surgeon (from hernia of the umbilical cord repair)- The site looks great, unfortunately it didn't close completely inside so more than likely she'll need another surgery to fully correct it.  He will see us back in the spring to see how it is doing, but it shouldn't bother her at all in that time.  He said the surgery will be done on an outpatient basis and there won't really be a new scar because they will do it right near the previous surgery site.

Ultrasound/bloodwork- These both went as well as can be expected.  She did really well with the 4 hour fast required for the ultrasound and was pretty relaxed during the ultrasound.  She cried when she got her blood taken.  :(  Luckily she calmed down pretty quickly afterward.  I did receive a call yesterday from the nurse for her geneticist saying that her AFP levels were trending down- on October 22nd, they were 833 and on November 9th, they were in the 500s (I forgot the exact number).  I haven't heard any results from the ultrasound, but I expect it to be clear with the AFP numbers we received.

On Monday the 19th, we see Julia's pediatrician for her 2 month well baby visit.  It is hard to believe she'll be 2 months old on Friday!  Assuming she stays healthy and nothing comes up, Julia will not see any doctors for the month of December - and January should only be a well baby visit!  I don't think I'll miss seeing all of her doctors for that time.

Well, if you made it through all of that, I'm impressed :)  Have a great day!