Evelyn and Sonya and Sonya's New Leg!

Sonya met up with her girlfriend Evelyn this past week at a yummy Italian restaurant! Evelyn is just a little over 2 months older than Sonya and she too has the same type of OI that Sonya has! Evelyn lives just about 20 minutes away from us, which is really awesome! Given that OI type 3 is pretty rare, it's quite amazing that Sonya has a twin just a few minutes away! The cuties had a great time pushing Evelyn's cool race car back and forth across the table and sharing the butterfly toy that Evelyn gave Sonya for Christmas while their mommies chatted away! The two little monkeys enjoyed standing in their mommies laps and jabbering about animal noises! The food was great! I definitely want to go there more often! I can't wait for our next get together! Guess what else?! Sonya and Evelyn are going to be on the same PAM cycle, so we will be hospital buddies our next visit! Evelyn has a port, so maybe she can show Sonya hers before treatment time to ease Sonya's fears! Here are some pic's of the two peas in a pod!

Sonya is totally making a funny face in this one!

This is my favorite! They are just too cute together! I'm so glad that Sonya and Evelyn will have each other for support and understanding as they grow up! I can already see all the fun they are going to have and possibly the trouble they will get into together! Hehe!

Ok, on to the leg... Sonya had her short leg cast removed today after almost 4 weeks of it being on!!! She had x-rays taken and they confirmed that she was good to go with no cast! Yay!!! She is a bit more bowed though... :(

With Sonya's type of OI, every time she fractures her bones heal bent, bowed or angled. They don't ever heal straight. The only thing that will fix the bowing is to add rods for support and straightening. Sonya's bones are not ready for this though as they have not had enough PAM treatment. If the doctors were to try rodding her now, her bones would just crumble. So as anxious as I am to get her rodded, we will have to let time and PAM work their magic first! It's better to do it right the first time!

Her left lower leg uncasted.

Curvy and bumpy tibia and fibula.

Q: Can you tell where the break was?

A: Right above the ankle!

Happy as ever playing her sister's guitar!

Thank you all for your prayers, love, hugs and support! The arm cast is scheduled to come off on January 4th the day before port surgery!

No PAM, Need a Port

Hi everyone! Thank you all for your prayers, hugs, love and support! We have been soaking them up!

Unfortunately, this week is not getting any better.... Just when I thought things couldn't be worse, Sonya's PAM treatments did not go well.

We went in yesterday for day 1 of treatment and they had a ultra hard time getting her IV placed. After having the IV team come in and give it their best first, we went up to the PACU to have them give her some Versed (a medicine sprayed up into the nose to reduce anxiety and mellow Sonya out a bit) and have their anesthesiologist give it a try. Even with this dose of chill out stuff, Sonya was still on to our intentions and tried putting up a good struggle which made things even more difficult. She doesn't know her own strength! The anesthesiologist had a hard time finding any viable veins in her hand. The only good one left was accessed just on Saturday for her triple surgeries and it was just too bruised to use, so she tried once in a "maybe" vein near her thumb. It blew out and she moved on to the foot. She tried once, was unsuccessful and then told me she would only try once more and that would be it. Luckily she managed to get a vein near Sonya's ankle and they taped her down good and covered it well so that Sonya would not disturb it. This whole process took about 2 hours!

Well, she got day 1 of her treatment but is skipping days 2 and 3 because her IV fell out sometime before we got into duPont today. They went to flush her IV and it leaked all over, indicating it was out of the vein. They tried manipulating it back into place, but it was no use and they blew out that vein. They tried the whole Versed technique again today but it was unsuccessful leaving Sonya with no good veins.

We knew that port surgery was eventually coming, we were just hoping to avoid it as long as possible. We don't have a choice at this point. She needs her treatment, but we can't keep poking her like this! We initially thought that we would have to have Sonya get her port within the next 10 weeks, before her next PAM treatment, but since they couldn't get an IV placed again things changed. Now she will be scheduled for surgery within 3 weeks from today. Ok, starting to really stress now!

While we were in today not receiving day 2 of PAM, Dr. Bober sent in the surgeons to talk to us about the port surgery, risks, benefits... We will have a pre-surgery appointment to discuss more and sign the papers. I do feel better after talking with them. The risks of infection are not so high when it comes to Sonya, as she is healthy in most aspects. For example, I will compare her to chemo patients who typically have ports. Her blood cell counts are correct, unlike a chemo patient, so she can fight off illnesses and infections better. She also won't be accessed as frequently as a chemo patient, just once every month for flushing and then once every 10 weeks for treatment (we are now going on a 10 week plan). So the chances for an infection to occur are less often. I am worried about clotting, so I will read up more on that. She will be getting flushed once a month by an in home nurse to prevent the clotting, but it makes me super nervous still.

Dr. Bober also sent in Dr. Kruse (Sonya's ortho) to check out her casts to see if he could lessen our travels a bit by skipping this Fridays appointment with him. Kruse thinks her leg won't be ready to be uncasted at that time so he bumped our appointment to Monday. He checked out her arm cast as well and agreed that it was too snug and that she was swelling too much! So he sent us for xrays to double check her elbow was still set and then sent us back to day medicine where he had sent in the casting technician to bivalve it (like a clam). She cut the cast on each side with the scary loud saw that Sonya is completely terrified of, pried it open slightly and then wrapped it with coban (sort of like a thin sticky Ace bandage) to keep it stable. Sonya seems more comfortable with it.

So today was productive, just not what I had expected. We are bummed that she did not get her full treatment and I really hope this doesn't mean she will be at higher risk for fracturing again. Dr. Bober doesn't think she will have any of her chronic pain coming back by missing most of this treatment and going off schedule, so that is good. As for the fractures, they happen anyway at anytime so I wouldn't be surprised, but I'm praying that isn't the case!

I am supposed to be hearing from the surgeon by the end of this week with a date for surgery. Dr. Bober is going to try and coinside the rest of Sonya's treatment with the surgery date, which some OI kids have done before, it's do-able! I'll update you all when we have a date set, until then prayers please!!! Thank you!!!

Arm Trouble

Hi Everyone! First of all, let me start off by thanking you all for your sweet comments on my last post "Christmas 2009 Photo Shoot". I love receiving comments on my photography and the girls cute coordinating outfits! Ok, most of you know but, this week is starting out a little rough! Here is the full crazy story! Friday afternoon Sonya was sitting on the hallway floor playing with a grocery store shopping bag, tossing it up and catching it, as Ian had just come home from doing the food shopping. Ian and I had started to put away the groceries and that's when it snapped. Sonya shrieked and screamed out "the scream", the one that comes when she is broken. "The scream" is something I have come to recognize within seconds now. It seems that Sonya lost her balance and tried to catch herself with her extended arm to the side. Unfortunately, it didn't support her falling body and it gave way.

I dropped the box in my hand and ran to pick her up. For some reason this gut reaction to pick her up just overcomes me! I have to start being a bit more aware and not let my emotions get the best of me. It's better to keep her where she is and not move her until I assess the situation. I carried her into the family room, laid her flat on her back and watched her movements. Right arm was swinging wildly... definitely not broken. Left arm was limp and frozen still and there was no movement in her right leg. I started with her leg after seeing her toes pointing down and her leg straightening stiff. No break in the leg.... onto the arm. I checked her humerus (upper arm) first, felt no bumps or added heat and heard no louder screams so I traveled down the rest of her arm. Screams got louder at the elbow. I skipped over it and went down to the radius and ulna (lower arm). I definitely felt a hot spot on her lower arm and a slight bump that was not there before... Ok, broken ulna or radius, not sure about the rest. I was worried because she didn't bend her arm at the elbow like she did when she broke her right arm back in March. She also didn't react well when her arm moved at the shoulder, so I worried about that as well.

The very first thing I do every single time we have a break is MEDICATE! I had Ian grab the "break kit" and pulled out the meds bag complete with the heavy duty stuff, Motrin, Tylenol and measuring syringes immediately. We dosed her with Tylenol with Codeine for pain and then spasm and anxiety control Diazepam. Then we moved onto splinting for stabilization. I splinted her at first using OCL (kind of like plaster casting material), but I sucked at it and I ended up making it too wet, so it wouldn't dry quick enough. I chucked it and dumped out the rest of my supplies in our "break kit". I whipped out the SAM splint that the EMT gave us from when Sonya broke her left femur (thigh bone) back in June. Wow! Is that splint the most awesome splint ever! I cut off a small slab and molded it quickly into a preliminary shape, slipped it under her arm, fit it the rest of the way carefully and then wrapped her and the splint up with an Ace bandage (the self sticky kind with no clips, also really awesome). Pinning her arm to the side, I had Ian watch her while I packed our bags.... another thing to add to the list of "should haves" all prepared ahead of time at all times. We just never know how long we might be in the hospital. Having to go by myself with Sonya most times, it helps to have all her basic needs plus some snacks on hand, a travel water bottle filled, and oh yeah, deodorant, toothbrush and paste, and a hairbrush at the least for myself!

This next thing may sound really crazy and weird and even selfish to some of you reading, but this kind of thing, broken bones that is, is a constant in our lives. It's common and frequent, typical and almost a weekly errr...at least a monthly event for us. I went upstairs to take a shower. Yes, I did. I needed one, I knew I would not have one until at least the next day and damn it all, I really wanted one! So, I went and took one! When Sonya breaks, we go to the hospital and they confirm it with an xray and then they splint her again... just undoing and redoing what I already had all set and then they send her home telling me to go to duPont to be casted the next day. A 30 minute shower would not screw up the whole schedule or put Sonya in any danger of not being treated immediately. This is of course the case with THIS kind of fracture. If it were something serious, like her head or neck or spine or ribcage or clavicle........ then obviously I would not have taken the time time to even pack a bag, we would have left immediately.

When we arrived at our local ER it was absolutely empty! We were in complete luck and we got taken into triage right away and then back for xrays immediately following. The xrays confirmed a broken ulna (lower arm bone), but it wasn't a complete through break. I can imagine this would be very painful having the bone rub against bone with no slight space between. They took images of her shoulder and her elbow and were concerned for both. We were discharged after being splinted and handed a prescription for more pain meds and we were told to call duPont for casting the next day. We went home and slept downstairs, Ian and I on the couch and Sonya on a small soft mattress pad on the floor where I could keep an eye on her.

Since it was now Saturday we had to go to the ER at duPont since there is no clinic on weekends. We headed out bright and early at 7am and arrived just after 8am. We sat in our little ER cubicle for an hour before even a nurse came in. She checked Sonya's vitals but not her blood pressure (BP). We do not do BP's on Sonya for risk of fracture. Sonya was given some Motrin at this time as her Tylenol with Codeine had run it's course by this point. In my head but keeping my cool: Motrin??? That's all? You're just going to give her Motrin? Her freakin' arm is broken!

Another hour passes before we see anyone! Sonya has been furiously freaking out the entire time and her heart rate must have been through the roof as her face was beat red and she was gasping for air! In my head but keeping my cool: What the hell! Are they at least going to give her some more Motrin? Where the hell is the on call resident what's his face and when the hell is he going to get his not busy butt in here!

I flag down a nurse. Literally! She says she has been paging the on call resident but he has been stuck in the OR (operating room) the whole time. In my head but keeping my cool : What the hell? Well, I hope he will be ready to operate again real soon, cause I'm going to break one of these nurses arms if they don't get my baby some serious medicine ASAP!

"Can we at least have her xrays reviewed (the ones from the local ER that I brought with) and get her something stronger than Motrin? She is in obvious pain and we would know what we are dealing with after looking at her films. Then once the on call gets here he can cast her up so we can be done." I quip. She followed my orders. Amazing! Note to self : Next time I am totally speaking up from the get go!

Of course 10 minutes later after the xrays are reviewed, the on call doc finally shows up and tells us that her shoulder is still questionable, her ulna is definitely fractured and her radial head (elbow) is most likely dislocated. He wants more xrays and we go for them. He decides to 15 minute sedate her (mini anesthesia), and try the first surgery (manual closed surgery, no incision) to pop it back into place, set the ulna and cast her. He thought he had it good, but the xrays later showed that the radial head popped back out! Surgery #2, same thing happened again! According to her films this time, she appeared to be off by a few millimeters, but if it was not set perfectly she would continue to have recurring issues with it her whole life. The next thing they wanted to try was a full sedation (anesthesia) fluoroscopy, where they inject dye into the area and do an arthrogram (live xray) while they perform surgery. This dye would help distinguish bone from cartilage, tendons and muscle tissue. OI complicates the whole thing. Sonya's bones and joints are deformed and her connective tissue disorder (lack of and not properly functioning collagen) obscures what is bone, what is cartilage and what is tendon or muscle in some parts of her body. This was evident while looking at her elbow xrays. Some of Sonya's joints have different positioning than the average person and this also was very evident when it came to her elbow. This makes it very hard to tell if her joints are correctly positioned (just right for her). If the fluoroscopy did not work they would have to move on to an MRI and open surgery (incision made).

We went upstairs to the OR for the fluoroscopy and I nearly started to cry as they sedated her yet again for the 3rd surgery in the same day. She cried and screamed for me before falling asleep, ripping at my heart strings! I waited impatiently in the dark and empty waiting room. Snow was starting to fall outside. My cell phone was battery dead, although even if I had a charged battery (bring the charger next time) there was no reception to speak of on that floor anyway (been there, done that). So I tried to read my book unsuccessfully and tried to write up my Walmart shopping list. I got as far as diapers and wipes and then I totally quit. Exhausted and stressed at the same time my shoulders grew tense as I waited. Finally about 45 minutes after I last saw Sonya a nurse came to get me and took me back to her recovery cubicle. Sonya was just beginning to stir when I came in so very happy to see her in a pretty new red cast all ready to start healing!

I wish I could say that Sonya was happy... When the anesthesia started to wear off she instantly became the Incredible Hulk! I swear she was stronger than I have ever felt her before! She was grunting, screaming, growling and swinging her uncasted fist and both legs (one casted) with intent! I asked her if she wanted me to pick her up and she was indecisive. After holding her for 2 minutes I decided it was best to have her lay in her bed or I too would be getting casted that night! I'm serious! This girl was ticked and she had every right to be! She was soothed shortly with the promise of food and managed to eat two popsicles and drank a whole cup of apple juice right there in the small OR recovery room!

We were then rolled back to a shared recovery room (the last and final straw that resulted in us getting the heck out of there ASAP). Sonya was completely at her full strength at that point. Feisty would be an understatement, this girl could probably win a bar fight with a 200lb+ angry drunk man! I tried shushing, humming, kissing her forehead, distracting her with Finding Nemo on the hospital's TV and various loved toys and books, and of course her blankie and "chewby" (pacifier), but to no avail. I resorted to laying Sonya flat on her back on the hospital bed, straddling her with my legs at her sides slightly restraining her as I sat there and waited for her to calm down. Sonya has the type of personality that any kind of talking down or attempt of calming her down in any other way just ends up infuriating her.... hmmmm... Stubborn! So we sat there and I stayed quiet, waiting. She finally soothed herself and half slept, half watched Finding Nemo.

The people in the shared room with us were ridiculous! Their TV was blaring some sports game and the woman was talking so freaking loud over it to her teen aged daughter who also had a broken leg (so they completely should have understood how it felt for a toddler in this case and could have been much more considerate). The husband was munching on his portion of their delicious smelling take out food (not gross hospital stuff and I hadn't ate anything since 2pm... it was now 10pm) and then he proudly and loudly belched as he continually grunted in satisfaction. Un-freaking-believable! With every boisterous laugh or deafening explosion of a burp Sonya would jump, startling herself and in turn ensue crying and distraught. Ugghhh! We needed to get the heck out of there!

Since Sonya was still hooked up to her IV and was receiving fluids, it didn't look promising. After an hour I called the nurse in, no longer able to take it anymore. I asked her when we could leave. She was under the impression that we were staying the night. I told her "Oh no! We were told by the doctor that we could leave tonight as long as I felt able to drive after being here all day and considering the storm outside. (Which was completely true, I would never fabricate a story even if the people on the other side of the curtain were astonishingly obnoxious!) I can totally do that! Sonya will feel much more comfortable at home! How much more fluid does she need?" She told us that since Sonya had the two popsicles and juice in the recovery room she could unhook her and get us discharged right away! Whoo! If I hadn't said anything we would have had to TRY to sleep next to that outrageous racket! So after getting Sonya disconnected from the IV, I had a wonderful time (insert sarcasm) getting my double casted Sonya into a some crazy tube sock involved ensemble. I wrapped her up in a blanket, used the stroller to cart the bags (isn't that what it's for anyway?) and headed out through the ER loving every "crazy lady walking her two limb casted baby out into the snow storm with no coat" look that I received on the way. Ugggghhhh! Ignorance!

After loading Sonya and our stuff into the cold van and scraping off the iced and snowed up windshield as my hands froze, we headed out on our way home. It was uneventful. Thank God! I was so over the top tired and just couldn't wait to be home again! Sonya slept the whole hour and a half home as I drove so paranoidly slow (50mph in 65 territory) the whole way. Turning into our neighborhood I stopped and looked around at the 4 inches of snow covered ground, pulled out my camera and took a picture of the beautiful white snow laying peacefully on each branch of the leafless winter tree that stood stark against the pitch black night. Ahhh! Home sweet home!

Ok, sorry this was so super long! It was a very long day and night! As some of my family and friends have pointed out, Sonya and I have an OI marathon this week! We are gearing up to head out back to duPont for Sonya's PT evaluation on Monday as well as her PAM treatment Monday, Tuesday and Wednesday and then we will go back on Friday again for a 3 week cast check up on her leg as well as a 1 week cast check up on her arm! I am just wiped out thinking about it! Thank you all for your prayers, love, hugs and support through all of this! You guys are the absolutely best and I am truely blessed to have you all in our life!

Side Note : All the shoulda', coulda', woulda's in this post are not me beating up on myself, but rather notes to myself for making the inevitable next time a much better more efficient experience.

Christmas 2009 Photo Shoot

So, here are the best shots that I managed to get of the girls on our Christmas 2009 photo shoot! I took the girls to one of the many historical covered bridges that we have here in Bucks County PA. I was inspired by my high school friend Jenny and the portraits that she had done a little bit ago by a great photographer. I think she had hers done at the same bridge! Anyways, it was a nice day with a temperature of 62! The crazy thing is, there was a huge windy torrential downpour last night! You gotta love this crazy PA weather! I can't believe how well Sonya's cast was hidden with her black stockings!

Our picture for our Christmas card this year!

I love how Sonya is looking up to her big sister Zoe in this one!

Zoe and Akela running the length of the bridge!

Akela checking out the wood work.

I caught Zoe in a moment of peace.

I love this picture of Zoe! She's such a natural!

My 3 gals on top of the stone incline.

Sonya just realized that she is on top of the stone incline and that is the end of pictures for her!

Hanging by the pretty trees.

I think Akela is picking at the moss here!

I got your hat!

This heart that was drawn on the inside edge of the bridge was one of the things that captured my eye when I was looking at Jenny's photo's and I knew I wanted a shot there!

Can you feel the love?!

This is the South Perkasie Bridge located in Lenape Park, Perkasie PA.

Twelve covered bridges are all that remain of the 36 built in historic Bucks County, Pennsylvania. Many reasons are advanced for building covered bridges. Some say they; provided shelter for the traveler. Others say that they protected animals from their natural fear of crossing water, while others say that the roof kept winter snows off the flooring. The more romantically inclined called them "kissing bridges" or even "wishing bridges". Actually, the roof was designed to protect the supporting beams on either side form the ravages of the weather. When thus covered, the bridge had a much longer life expectancy.

All of the bridges which still stand in Bucks County are built with a lattice-type construction. First developed in 1820 by Ithiel Town, a New Englander. This consists of a series of overlapping triangles with no arches or upright beams. Such lattice work could support a bridge 200 feet long.

The worst enemies of the covered bridge are weather, fire, fast moving vehicles and neglect. Fortunately, the historical value of these bridges was realized as they have been protected, preserved and in some cases reconstructed.

This is the only bridge in the county which does not cross water. It once spanned Pleasant Spring Creek, but some years ago it was condemned by the County. The Perkasie Historical Society led a community project which eventually saw the bridge moved to the safety of Lenape Park in Perkasie. It was dedicated as feature of the park on August 15, 1959 and Congressman Willard S. Curtin was the principle speaker.

The bridge is 93 feet long and was built in 1832 of oak and pine. It is one of three of the oldest remaining covered bridges in Bucks County. A famous sign on the end of the bridge reads "$5.00 fine for any person riding or driving over this bridge faster than a walk or smoking a segar on"

Biannual Checkup and a Visit With the Greenes!

On Tuesday morning we headed on down to Baltimore for Sonya's biannual checkup with Dr. Ain. Sonya had a series of xrays done so that Dr. Ain could get a good idea of what's going on with her spine. Sonya has a combination of Scoliosis and Kyphosis and we were quite worried earlier about her C1 C2 instability and Basilar Invagination.

Basilar Invagination is also referred to as Basilar Impression (BI). It is an upward protrusion of the top of the spine into the base of the skull. It results in neurological complications that are potentially very serious. Symptoms can include headache, double vision, imbalance and arm or leg weakness. About 50% of people with OI type III or IV have moderate or severe BI.

Dr. Ain still thinks that Sonya is in the OK zone for now and wants to see us back in 6 more months. He did see many more than 5 spinal compression fractures though. We knew she had 5 about 6 months ago...... now there is much more which means her spine is getting worse fairly quick. The Pamidronate treatment that she is on is supposed to go straight to her spine and strengthen it first before spreading through the rest of her body, so i can't imagine where she would be right now if she wasn't receiving treatment! Dr. Ain is going to be emailing me the total amount of compression fractures as well as the degrees of Kyphosis and Scoliosis once he finishes measuring the xrays and counting up the flattened vertebrae. I have noticed that I can now (in the past 2 months or so) feel Sonya's Scoliosis and Kyphosis curves. It's worrying the heck out of me, but nothing scares me more than Basilar Invagination!

Earlier we were considering not seeing Dr. Ain anymore and keeping all of Sonya's care at AI duPont. We have decided to keep Dr. Ain on board for several reasons after weighing the pros and cons. Our biggest reasons are :

#1. Dr. Ain is the only doctor who is seriously concerned about her back and is the only one keeping an eye on it!

#2. duPont is a children's hospital and Sonya will only be able to receive care there until she is 18 years old. If we stay involved at Hopkins then the transfer of care at that time would be a much smoother transition seeing as how they will have been following her from the start!

While we were there we got Sonya re-casted since she managed to wiggle out of her old cast on the car ride down to Hopkins. So now she is back to just green! We have a check up at duPont next Friday to see how her leg is healing and they will take off the cast again to xray. So if she has to be re-casted again it will be the 4th cast for the same break!

After our appointment with Dr. Ain we had a great time meeting up with the Greenes! Sonya's boyfriend Simon was as cute as ever! Both Sonya and Simon were a bit tired when we first arrived. For Simon, it was his nap time and Sonya was just exhausted after screaming her lungs out getting xrays and a new cast. After getting some chicken strips and french fries into their tummies they were a bit better and more social! Haha!

Simon gave me a great "cheesey smile"!

Munching on french fries!

They both were very interested in Sonya's tray release button for some reason!

And of course being the bully that Sonya can sometimes be, she shoved Simon's hands away and yelled at him "No!"

Which of course upset Simon! Sonya is such a heart breaker!

But, he forgave her! Ahh... young love!

Here are the three little monkeys all together! Jude is sooooo cute! He is such a good baby! I didn't hear one peep out of him the whole time! Looks like he is going to be one easy going dude!

Simon was very fascinated with Sonya's pacifier. Sonya calls it her "chewby" and she is completely addicted! We are having the Easter Bunny take them this coming Easter just like we did with the older girls who were also hopelessly addicted.

Looks like Sonya is giving Simon the "thumbs up"!

Can you believe these two have had eyes for each other for over a year now?! That's right! We all met for the first time back on November 21st 2008. Check out their first date here! Thank you for meeting up with us Jenn, Simon and Jude! Thank you so much for Sonya's care package too! She loves her Elmo book, Smurfette, light up gadget and "puffies"! We missed you Jason, but there's always next time of course! For more pictures check out Jenn's blog!