Curve Ball

Sophie had her first appointment with CHOP today (Children's Hospital of Philadelphia). They have a satellite office closer to our house than the big hospital in Philly, so we went there. The visit was to establish a relationship with her new ENT (Ear, Nose and Throat doctor). She had tubes inserted into her ears in March of 2009 and recently her pediatrician couldn't see them during an exam (no big surprise there as most docs can't). We needed to see if they were actually still there.

Like many people with Down syndrome, Sophie's ear canals are very narrow making it difficult to visualize the ear drum without a trained eye and special equipment. Dr. W took a look after clearing out a lot of wax and her tubes are still in place and look good. He surprised me by sending us off to audiology for a behavioral hearing test right there and then. During the last two auditory tests at St. Louis Children's Hospital the audiologists said her hearing appeared normal in the conversational level but showed a mild loss in the lower decibels. (in these tests the audiologist trains the child to turn to a stimulus - a light and toy that makes noise when they hear a sound. Then the audiologist just plays the sound to see if the child will turn to it for the reward of seeing the toy, etc.)

Today the tympanogram showed that the tubes were clear (a good thing) especially as she has a cold though it hasn't migrated to her ears (knock on wood). However the behavioral hearing test showed mild hearing loss in both ears (more loss in the right than the left). The loss is in the conversation range and the higher tones which is different from what the last two tests showed at St. Louis Children's Hospital. Of course, this was done through a behavior test with a little girl with few words and a developmental delay so they can't be certain about the accuracy of the level of loss without further testing.

In February we will head to Philly to CHOP for a sedated ABR to confirm or rule the loss out and to find out about each ear specifically. If the loss is confirmed, hearing aids may be the next step. However, I don't want to get ahead of myself, so I'm trying to reign my thoughts in about that until we know for certain.

My blog has been quiet lately. Writing daily in October really burned me out and I have been working through a lot of old and new personal ~stuff~. I've written posts I haven't published. It's always a trick to know what to share in such a public venue. Blogging has been my outlet and not feeling motivated to write or comfortable in sharing has been something I've been trying to work through.

A recent development is that Helena (who is 18 months old) has surpassed Sophia in vocabulary. While I knew it would happen eventually, adjusting has been...interesting. Mark and I have also both been very homesick. We are saving to buy a house, so we decided not to travel back to the midwest for the holidays. Thankfully family is coming to us. Mark's cousin Greg is staying with us for a while until his new place and job are ready in Jersey. Mark's parents are coming up in the next few days and later next week his little bother will be here. It will be a blow up air mattress slumber party and we're very excited.

So, tonight as I digest the latest curve ball that we've been thrown, I'm trying to keep it all in perspective. Hearing loss is something that we can work with. It is not completely out of left field given Sophie's DS, though it did take me by surprise. I will try and focus on being thankful for our awesome health care and doctors. I am thankful for the opportunity to catch it early, for those with DS who have come before us who have taught us to screen for things like hearing loss at such an early age, and for the amazing technology to help if her hearing loss is confirmed.

our home as taken by iPhone

an avalanche of Christmas bears (2 from 1987!)

Speedy, our hotly debated Elf on the Shelf (but that's another post)

Decorating the tree as captured by the iPhone

MY NEW SEASONAL ANTHEM!

Ear Tube Follow Up and Audiology

Most importantly, it was a good visit. In and out in 1 hour to ENT and audiology is a world record. Sophie's ears look good and the hearing test went well. The results are pretty much the same as in October. Her hearing is pretty good with a mild loss in the low pitches. Follow up in 6 months.

The trip, however, was another story. We got into town with a smelly engine and without a stroller with which to haul our stuff. While I took Soph to the appointments in a borrowed hippo shaped wagon from the hospital, Mark and Alexander found a shop within walking distance of SLCH. Appointments were done by 12:30 but the car hadn't been checked out yet. We spent the day between the 2nd floor entry way with the trains that are mounted around the perimeter of the ceiling and in the cafeteria.

We heard from the shop that the AC compressor was bad and they did not recommend trying to drive it home. If the belt broke, it would possibly cause serious damage to the engine and we would be stuck on the side of the road. Mark's dad concurred. They couldn't get to it until today, so we had to decide if we would be staying overnight or renting a car to get home. Staying would have meant rescheduling ST, the tub surround repair (major leak into the basement from the main floor) and Mark missing 3 classes. That and we had no supplies or Sophie's meds or extra food out for the cats. Thankfully, RK and Julie came to the rescue and drove in from St. Charles. We are borrowing RKs car and returning it, probably Wednesday, which meant that we were able to drive the 3 hours and be home last night. Thank you RK! On the way home, driving through the rain, Alexander had a potty accident and he ended up with a brand new pair of St. Louis Cardinals pjs. I was fully convinced that my water would break (as we're not technically supposed to be travelling out of town) and well, it would have fit the theme of the day.

I spent the day hysterically laughing to the point of tears, which is dangerous given my near full term pregnant status. The car could have broken down in the rain on our way, causing us to miss our appointment and stranding us, who-knows-where. It didn't. We thought the over due oil change may have been the problem and were planning to just get an oil change. Had we done that, instead of taking it to a shop, it would likely have broken down on the way home. Had we remembered the stroller, Sophie might not have had such a good nap in the cafeteria, in the bed we made for her in the hippo wagon. She doesn't nap well in the stroller. Had we left right away after the appointments, I would not have had the yummy onion rings that I love. As our little comedy of errors continued to develop, I couldn't help but see quite clearly how much worse it could have been.

2nd floor entry, trains

Cheesy post-nap grin

Alexander working hard on Noggin.com in cafeteria

Sophie's Ear Tubes

It's been a full week since our trip to St. Louis Children's Hospital where Sophie got tubes in her ears. Grandma Paula hung out at home so that Alexander could go to preschool. He did just fine, but I had a harder time. It was my first night away from him.

We got in around 9pm and settled into our hotel. Sophie slept most of the way there, so she ended up staying awake until 11:15. That was OK because I could give her food until midnight and then she needed to fast.

SLCH is beyond good at what they do. Click here for a previous experience describing the care you receive there. We arrived at Same Day Surgery by 7:15 and by 8:34 she was headed back to surgery (scheduled for 8:30). I love it when a procedure happens on time, especially when fasting is required.

It was really fast and she was done 30 minutes later. Super cranky and out of it, we tried to comfort her and get her to drink apple juice. More quickly than I imagined possible, we were leaving. She did vomit up snotty juice as soon as we left and were in the car, but after that, she was better. It took a while to fully come out of sedation.

We met our very good girlfriends at a mall. (Why hang at the hospital when we can shop?) Plus, they had a Stride Rite and our only local store that carried them closed. Sophie needs a 4XW, which you just can't get anywhere. RK is a super mom, looking gorgeous, lugging 2 around as if she's done this forever, and does well multi-tasking. Click here for their pics of our meet up. We were so happy to finally meet Kinlee and see our old pals.

Mark always notices the quirkiest things. This sign at Dillards disturbed him, so he snapped a shot.

The next day Soph needed Tylenol, but has been doing great with zero drainage. We go back for a follow up and appointment with audiology in 4-8 weeks (which we will try to coordinate with her follow up eye appointment). So far, so good.

Tubes

So, we're chillin in the hotel in St. Louis. Early tomorrow morning we check in to SLCH so that Sophie can get the first set of tubes in her ears. I've added twitter back to my sidebar, but as this is such a quick procedure that I may not have time to add anything. Afterwards I get to gobble up the 3 week old Kinlee! (oh, and see her big sis and mommy). I can smell the sweet new baby smells already!

On another note, after being on the waiting list for more than a year, we are going to receive Respite! That's 15 hours a month (40 hours quarterly) of help watching Miss Soph so that I can get housework done, run errands, or (gasp) get out of the house!!! Our regular sitters are under 18, so we'll probably use some of the respite workers that DSC already works with (many of whom are special education students). Our intake meeting is this week and then we're good to go. This is a real blessing for our little family.

Lastly, the elevator in our hotel has a button that simply says, "earthquake." hmmm? Do I really have the power to cause an earthquake simply by pushing that button?

Eyes and Ears

Sophie's 11:30 appointment with ENT/audiology finally got under way today at St. Louis Children's Hospital around 12:45. The nurse held her down with a vice grip so that the ENT could get a look and clean out her waxy ears. She screamed so much that there were snot bubbles coming out of her nose and mouth and capillaries broke all over her little face (see red spots in pic above). He got a good look in her right ear and saw fluid and no movement of the ear drum. The left ear was even more waxy and Sophie had had enough so I asked for a break. He didn't get as clear of a look, but decided to stop as he had made his decision. (The left ear also had "some white" in it.) So, she's getting tubes in both ears at SLCH in 3 weeks. Audiology was cancelled for the day as it would be pointless now and they check hearing 4-6 weeks post surgery anyway.

Then, the eyes. Sophie's Strabismus surgery last May on her left eye was related to superior oblique muscle palsy. To 'fix' it, the surgeon cut and moved the opposite muscle to weakened it so that they would be even (layman's terms). This muscle effects more of the up and down movement of the eye. What Sophie has now is also Strabismus, but of the (bi)medial rectus muscle, or those that effect the left to right movement. As this Momologist suspected and insisted they check (though I'm sure they would have anyway), this time BOTH eyes are affected. Her eyes are not turning in severely at this point and her vision is good and pretty much equal in both eyes, which means her brain is not favoring one over the other. They're calling it intermittent esotropia. We were instructed to come back in 3-4 months or earlier if it gets worse. Hmm. I wonder what we might be doing around May 10th (Maybe's due date), and June (a possible move depending on the job situation)? So we haven't scheduled yet as I've got to figure that out.

We didn't see our regular opthomologist today as she was in surgery. Instead we had a resident. Nice guy, but I've got more questions than answers and will be calling to talk to the doc tomorrow. The resident said that this is not something that eye exercises or patching will help and that 'possible' bimedial rectus recession would be needed. However, once you start moving more than one muscle, you can end up with 'weird' things happening.

I know it could be much worse news. I will adjust to this new information and move forward. In the meantime I had a hot fudge sundae on the drive home.

Also, our dentist had the misfortune of calling to reschedule Alexander's appointment for Friday. This would be the 4th appointment in a row that they've rescheduled just days prior so that the dentist could attend a meeting or training. Guess what? I fired them and politely but firmly explained that they clearly do not value their patients' time. So now we need a new dentist.

On a wonderful note, it was 66 degrees in St. Louis and Kinlee was born today! I won't steal her momma's glory, so I'll hold back from revealing the details. We had planned to get a peek, but with our sickies, we opted to drop off some goodies with RK's sister instead. We'll see them in 3 weeks when we go back and I won't have to fight off the crowd! Congratulations to the family!

You're Fired

When was the last time that such a thing was wonderful news? I'll tell you when. When we were told not to ever come back, when we crossed an 'ologist off of Sophie's list. When Sophie was born there were 3 findings regarding her heart including a small muscular VSD, PFO and small PDA. None of the findings required any action beyond monitoring. Last February, Sophie had a repeat echo cardiogram that revealed that she only had a small PDA. Yesterday she had another echo and I am overjoyed to announce that the small PDA has closed on its own and we no longer have need of a cardiologist! That's right. Dr. Heart fired us and we couldn't be happier.

Other health updates are that Sophie is working on tooth number 8, a lower left molar. Next week we travel back to St. Louis Children's Hospital for an appointment with her ENT and audiology. This time they plan to isolate her ears to find out how each one is hearing. We have also been worked into the schedule with opthomology. After Sophie's last check up, we were told to come back in a year. However, the eye that she had Strabismus surgery on has begun turning inward and upward slightly, once again. I already knew that it is not uncommon to need repeat Strabismus surgeries, but am hoping that won't be the case.

In archiving my videos recently, it struck me how much she has changed in her 19 months on Earth. She's no longer my little baby. She's getting bigger and doing more every day. This update is not meant to be a comparison or brag and it is not my wish to upset any other extra special moms who I know love her. I'm proud of Sophie's accomplishments and I know you are too, just as I am proud of the little ones I've come to know and love.

Sophie has added the word 'boo' to 'peek.' Her favorite words are Daddy, baby, and Bubba (Mom/Mama comes in much further down the list). She waves and says 'bye bye' consistently and in the right setting. She even shouts it if the person she's greeting is far away. She's a millisecond away from cruising and pulls to stand like a champ. She could set world records in speed crawling. She kisses with puckered lips and sound vs. her original open mouth slobbers. She makes car and train noises when playing with Alexander's toys. She's developed a giggle and sense of humor that's contagious. Sophie nurses once at night and I expect to wean her by the end of the month. Straw drinking was a long and wholly worthwhile learning experience and without her ST, I'd be tandem nursing! She's great at putting toys 'in' (even when its other things like Daddy's slipper 'in' the bathtub or a ball 'in' the toilet). She has a great attention span, even with new toys, and gets very focused and intense. She seems to understand questions, like "where's Daddy?" because she often points with her index finger (though not every time). She often complies to requests like, 'hand me the toy.' She loves books and anything her big brother likes. In fact, she thinks her brother is the coolest guy around. She's such a social butterfly and she draws people to her where ever she goes. She also has developed a little princess attitude when she doesn't get her way, though it's so cute, its hard to get mad when she's being a stinker. In a word, we think she's pretty great.

(blog note: in the new menu bar, the VIDEO and CONTACT sections have been updated)

Avenue of the Saints

1. Sophia's biopsy for Hirschprung's disease was negative!
(her new diet with more fruit, Yo Baby yogurt, and no bananas is working wonders!)
2. Sophia had surgery on her eyes today. (both eyes - tear duct probe, left eye - Strabismus muscle surgery) We are finally at home, resting.

We spent the weekend at a Bed and Breakfast in the Amana Colonies (Middle Amana) Iowa in celebration of M's little brother's graduation from the University of Iowa. After no internet access for days, and a huge change of plans, Sophie and I drove from IA City to St. Louis on Sunday by ourselves {on the Avenue of the Saints} and stayed overnight in the Parkview Hotel. As her surgery was in the morning we set our alarm and woke up in the middle of the night for a sweet potato picnic (as she was not allowed to eat after 1:30 AM). This morning RK and Braska Bear met us at St. Louis Children's Hospital and spent the day being our cheerleaders/gopher/chauffeur. They are staying with us for a few days, so its turned into a slumber party! We are blessed with wonderful friends. According to the docs the surgery went well. We won't know for 3 months if the Strabismus surgery was a success and we return in a week or so for a follow up appointment.

Meet Me in St. Louis Part 2

St. Louis Children's Hospital is really an amazing place. From the time you walk in the doors you are greeted with smiles. The security guard asks if he can help you find your way. Everyone is patient and they all seem to really care about the children and their families. Everywhere you look there are things to help take your mind off of the reason for your visit. On the outside of the building, there is a giant metal down spout shaped like an elephant's head whose trunk whisks the rain water away. The awning is a blue butterfly. Inside there are fish tanks, a magical ball machine, and colorful paintings on the walls, all done with the utmost attention to detail. A child's voice announces each floor in the elevators. Alexander's favorite place is the 2nd floor hallway where large model train tracks are hoisted around the perimeter of the ceiling encased in plexi glass. He's spent quite a bit of time there already chasing the 3 sets of trains that chug above his head.

On our first visit to Children's we saw 3 doctors and one therapist. Our time was spent in colorful office spaces and waiting rooms. The HD biopsy, however was a little different. For this visit Sophie was admitted and was fitted with a wrist band. We all needed name badges (Sophie's Mom, Sophie's Dad, Sophie's Brother). I expected to be taken into an office like the others we had been in. As Nurse Charlie escorted us, my heart stopped as a very, very premature baby was wheeled by in an incubator. My breath actually caught in my throat and I found myself choking up. I hadn't expected to see anything like that. I think I actually stopped walking for a second. Charlie gently touched my shoulder and whispered, "There but for the grace of God. Ok Mom, take a big breath in...now let it out. You've been there before haven't you?" Now Sophie wasn't that small when she was born, but she was in an incubator, naked, hooked up to tubes and beeping machines that look like fish finders.

To get to our room we passed by a busy nurses station (just like in our NICU) and saw lots of other children in beds with tubes and parents with worried looks and beeping fish finders (just like our NICU). I was surprised to feel so off kilter. It was a relief to get into our room and shut the door.

Nurse Charlie was lovely. She took her time explaining the procedure. She asked if we had any questions and let Sophie and I examine the instrument that they would be using to do the HD biopsy.

It looks complicated and scary, but only the silver part is inserted. The syringe-like part gets pulled back to create the suction and the trigger is pulled snipping off a small piece of tissue that has been sucked into the side of the silver part (hence the name, rectal suction biopsy).

Sophie's doctor was Australian and had a lovely accent and bedside manner. He took 3 samples and was done in a matter of moments. As the tissue is taken from an area that doesn't have pain receptors, Sophie barley fussed at all. We'll get the results in 7-10 days.

That night for dinner we visited Blueberry Hill in the Loop. M was in heaven as they had his favorite childhood pinball game "the Twilight Zone" AND Fat Tire on tap.
Alexander made lollipops out of cheddar cheese balls and then started eating the rest of his dinner with a toothpick.

On the way back to the car we popped into Vintage Vinyl Records and stepped back in time. M and I met in college while working for an independent record store. We both thought these stores had gone the way of the dinosaur and were so happy to find this place. As we perused the CDs encased in plastic shucks (those darn things broke many a fingernail back in the day) and records (as in actual vinyl - the only real way to listen to music) we were near giddy. The clerks with scenester clothes and bored expressions made me smile. There was even a live "performance" of local rap artist Ruka Puff promoting a mixed tape of St. Louis rappers. It was quite loud and the kids looked confused as to why we would be there. We figured a few minutes wouldn't do any real hearing damage. The best part was when M discovered a cash of Blowfly CDs. It wasn't just that they had at least 10 copies that cracked us up, but that they were filed next to Kids Corner CDs. {For those who are unaware, Blowfly's bio reads, "Before there was Kool Keith, Old Dirty Bastard or 2 Live Crew - before there was hip hop, for that matter - there was Blowfly, performing X-rated songs with a funky groove." I can't even list titles of his songs here. Goggle him, you'll see.} Only an independent store like this and the one we worked at stock this type of... art?

Alexander got a special treat as we came upon an old trolley car nearby which promptly became his favorite. M has decided that it would be amazing to get a job at Wash U just so he could shop at Vintage and stop by Blueberry Hill for the beer and games.

Then today was Sophie's pre-op appointment with opthomology.

Marlo retested her eyes and its true, her left eye is turning in a bit more than during our last visit (I thought so and should have trusted my Momologist instincts). But, she got even better measurements this time and we will proceed with Strabismus surgery on her left eye and nasal lacrimal tear duct surgery on both eyes on May 19th.

All things considered, it was not a bad trip.

Random Information

Tomorrow is Sophie's biopsy for Hirschprung's disease at St. Louis Children's Hospital. We're staying the night as she has her pre-surgery appointment for Strabismus and tear duct surgeries (scheduled for 5/19) with the opthomologist on Friday. Prayers welcomed, updates forthcoming.

The credit union finally reimbursed us the funds that were "withdrawn" in London. Bout time.

Today Sophie had her 1/month Developmental Therapy with Judy. We both agreed that she is a smart cookie. So is Judy. Plus, she has great toys.

According to my dear friend Amy, it's Wordless Wednesday, but rules are meant to be broken. That said, this dorky pic is a shot out to my BFF Darcy - Queen of the self portrait.

(post dental cleaning cell phone self portrait)

Meet me in St. Louis

Dilated Eyes - 1st eye exam

We went to St. Louis Children's Hospital yesterday to get Sophia checked out. We saw 3 doctors and a speech therapist.
Here's the skinny:
Sophia has misalignment of the left eye due to a weakened muscle that she's had since birth. She is so smart that she has figured out that when she tilts her head, she can correct her vision as she's likely been seeing double. She'll need Strabismus surgery (eye muscle surgery) and while they're in there they'll do a nasal lacrimal duct surgery on both eyes as she has had lots of yucky discharge for a while. Dr. C said, "I see she tilts her head." I said we started Physical Therapy for Torticollis around 6 months of age. She said she just recently gave a talk about misdiagnosis of Torticollis when it's Strabismus to a group of peds docs. PHARFIGNEWTON!!! The good news is that her vision is very good regardless.
Next, her new GI, who he says has spent a lot of years working and studying Hirschsprung's disease, is especially concerned that her constipation may be caused by this. She needs a rectal suction biopsy and then we'll go from there. I did a google search for him and Hirschprung's and he's listed many places. In layman's terms, this is when there are missing nerve cells in the bottom segment of the intestines. This causes the stool not to move all the way through the end of the intestines. Repair is done by cutting out the effected part of the intestines and reattaching it to the anus. (Her duodenal atresia was the uppermost intestine that was blocked and was cut out and reattached to the stomach.) In the general population Hirschprung's occurs in 1 in every 5,000 but with Down syndrome it is 1 in 75.
We're scheduled for the biopsy on May 8th and pre-surgery appt. with opthomology on May 9th. Eye surgery will be May 19th. We'll have to wait and see what the biopsy tells us about the next steps there.

For you detaily people; click on the bold type for information on Hirschprung's disease,
and on Strabismus, and on Nasal Lacrimal Duct Surgery