Tongue

 

I went to town this afternoon to run some errands. Just me and HMae and I was quickly reminded of exactly why we are going ahead with the tongue reduction surgery. HMae had fallen asleep on our errand run. I went into one last store toting her in her car seat. When a women commented on her sticky out-y tongue the very second we walked in the door. She was sweet and by no means did her words offend me but It has me wondering if HMae had a huge nose would people comment nearly as much? If only they knew what my sweet girl is going to have to go through in a few weeks. I would tell them but I always feel guilty weighing their hearts down. The truth is she doesn't just have a big tongue. Her tongue seems minor when in comparison to the other implications of BWS. She also has a much greater chance of developing childhood cancer by the time she is ten. She will have to have blood tests every six weeks to monitor her AFP levels. Ultrasounds every three months. Physical therapy, occupational therapy, speech therapy. Another possible surgery to correct her kidney function. At first, I could have crawled into a corner and cried for days. But I instantly realized none of that would do any good. We try to flip it to the positive side as much as possible. Yes, we have long car trips as we travel to CHLA but we also get to have our girly time! We make laps around Nordstrom as we scour the shelves for the newest headbands and bows. We rest our heads in comfy hotels. We have the best road trip buddies that keep us company during the long drive. We wear the cutest band-aids after blood draws. And don't forget she gets to wear the most adorable of bibs to catch all that drooly drool! It is what it is and we couldn't be happier to be on this journey with our sweet girl.

 

As many problems that this little tongue has caused and will cause in the future I must say I'm going to miss it! You know that feeling when you are looking at someone and you know there is something different about them but you can't quite place what it is. A haircut maybe? Shaved off a mustache? I feel like that is how we may feel after this surgery. This little tongue is so much a part of her personality. We have started to notice that as HMae gets older she is gaining more control over it and has started to make the funniest noises. As cute as it may be breathing and eating and talking and sleeping are much more important. :)

Road Trip

The in-laws have invaded! Ma & Pa Kettle as the Hubs and I refer to them parked in our driveway on Friday around 2pm with no intent to leave for two weeks. Living in Colorado, they traveled for two days to see their two adorable grand babies and us too of course. After a few days of playing with the kiddos it was time for Haddie and I to make our trip to CHLA. This time we were going to meet with Plastic Surgery. Graciously, Ma Kettle offered to come a long for the ride. Now 300 miles sitting in a car with your mother in law might seem like a nightmare for some. But again I am lucky. We had so much fun. We headed for Burbank where our hotel was but of course had to detour to IKEA! Duh. Poor Ma Kettle was a little overwhelmed by IKEA and all that it is but still had fun. Later we checked into our hotel then traveled downstairs to The Daily Grill where Ma Kettle treated me to the largest chicken pot pie one could ever consume. It was bigger than my head! HMae was such a trooper this trip. She hardly cried at all in the car and actually slept most of the way. Although she did pay me back by not sleeping at all the entire night in the hotel. Not sleeping is actually normal for us lately.
In the morning we traveled down the road a little ways to CHLA. A nurse came into the waiting room gathered us up and took HMae back to a room for some pictures. They propped her up on a Bumbo seat that sat atop a stool and took a few pictures to document her large tongue. After waiting for what always seems like an eternity(20 min or so). Dr. Hammodeh entered and chatted a bit with us about HMae's condition. He was knowledgeable and quick to respond to any questions we had without hesitation. Ultimately we decided to go forward with a surgery that will correct the abnormality that is HMae's tongue. I had decided long ago that we would not walk out of that office without a surgery date and I thought I would have to fight for it. I was wrong. Within about 5 minutes of conversing, Dr. Hammoudeh insisted we do this as soon as possible because waiting wouldn't do us any good. So we got it. June 15 is the day. You better believe we are terrified! But we also know that we have to do it for her. We promised her that we would give her the best, most liveable life we could provide.
Needless to say we had one very successful trip. Ma Kettle offered so much comfort to me. I just wish we could have her closer. And as for Pa Kettle, Koen is obsessed with him. He even makes Pa rock him to sleep at night. I think Pa Kettle is a very happy man!

A little about HMae's pending surgery...
It is formally called a Tongue Reduction surgery. It will be preformed by Dr. Hammodeh and his assistants. The surgery itself will take about two hours. HMae will remain in Pediatirc Intensive Care Unit for about 4 days. After that she will be released into a general recovery room. We are anticipating about a week in the hospital.
Please ask questions if you have them. I will answer them the best I can. I am going to tend to this blog as often as I can to keep everyone updated throughout our journey.

Much Love,
Missy Hughes

Why not us?

When life is troubling we often ask ourselves "Why Me?" I remember when we found out HMae would be born with a uncertain medical condition my husband asked this same question. "Why us?" "Why her?" I searched for the perfect answer to help him through it. You could call me an optimist. I often look at the sunny side of things because I believe that it allows me to live a happier life. Then I found it. "Why not us" I said confidently. As I began to sell him the thought. We can give her endless love. We can give her endless strength. We can provide for her. We can find the best doctors. We can hold her hand through uncertainty. We will find the prettiest band-aids to cover her blood draws:) We will do everything possible to give her the best life she can live. We can teach her and guide her. We are perfect for this because we are strong and even when we are not, we have each other.

Reflection

9.13.11

An ultrasound, one of many, but one that would change our lives as we knew it. At this point it had become quite routine.  But this day was different. On the eve of my Daddy's funeral, I lay in the doctor's office while my belly was covered in blue goop, waiting to see what Dr. Cedars evaluation was. And as it was, we had run out of amniotic fluid. Which meant an induction in the morning. So many thoughts raced through my head. I couldn't count them if I tried. I was told to walk over to the OB and let him know we needed baby girl to enter this world now. Easy enough right? I barely made it to the lobby before my OB's nurse greeted me. Dr. Haupt came in and said "So we're having a baby tomorrow?" It sounded good to me. I mean that was what we were here for, right? This ended up being one of those moments where life slaps you in the face and makes you realize that having faith in Him is having faith in His timing. This slap had happened a few too many times in the recent months. 

8 am:
 We were checked in and comfy, well for now. I couldn't stop thinking about him that day for obvious reasons. My heart was broken. He wasn't there.

1 pm:
 A few hundred people had gathered to celebrate the life he had lived. An ironic day it was. In life there is death and in death there is life. I have struggled with this more than some may know. Almost as if He said, I grant you your wish of a sweet baby girl but it comes with a price. I have healed that thought now because I know that everything happens with reason and purpose. Some we may never understand. But this is the way it is meant to be.

The hours that followed were filled with pain in more ways than one. A few visitors had come and gone but for the most part we were alone. Until she decided it was time.

8:48pm:
 One tiny push is all it took to bring her tiny little body into this world. Our first thought, how amazing it was to hold our sweet precious girl. Our second thought, her tongue was huge and seemed to stick out a mile long. We thought it was quite funny. She showed us very quickly what she thought about entering this world without getting to meet her Papa. Although, I know he was there guiding her though. Holding her every second before she got here to us.

The days that followed were some of the most uncertain days. She seemed healthy, was eating very well but the pediatrician had his suspicions. Too many physical characteristics were presenting themselves and he was uncertain of what it could all add up to. Ultrasounds, EKGs, scans, bloods were ran to hopefully figure this out. 

Months passed...

A referral to genetics landed us at Children's Hospital LA.
It wasn't our first visit as we had been seen by the Urologist prior to her birth for the known kidney issues. But this time we would walk out of there with a speculated diagnosis. Beckwith Wiedemann Syndrome. My head spun as words like malignant tumors, cancer, disease and risk were suddenly thrown into a conversation just as easily as THE or AND would be. A pile of paperwork and we were directed downstairs to have blood work drawn. Waiting for blood work, our good friend Mr. Wikipedia guided me through this disease. And so simply put, he said...  

Beckwith–Wiedemann syndrome is an overgrowth disorder usually (but not always) present at birth characterized by an increased risk of childhood cancer and certain congenital features.

 And then a phone call to the Hubs to inform him of what we had learned. He stayed strong for me on the phone but I know his heart sank and his head spun just as it did to me. I didn't sugar coat, I knew it would just hurt more later if I did.

Here we were with a chance of 1 in 13,000. Only 300 babies would be born this year with BWS. And it was this sweet tiny girl who got to be so lucky. Her smile molded by that big ole tongue would brighten up any room she entered.