3 Pretty Girls

No PAM, Need a Port

noreply@blogger.com (Melissa Swartley) — Tue, 08 Dec 2009 22:14:00 +0000

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

noreply@blogger.com (Melissa Swartley) — Sun, 06 Dec 2009 23:27:00 +0000

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

noreply@blogger.com (Melissa Swartley) — Thu, 03 Dec 2009 21:29:00 +0000

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!

noreply@blogger.com (Melissa Swartley) — Wed, 02 Dec 2009 22:28:00 +0000

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!

Catching Up

noreply@blogger.com (Melissa Swartley) — Mon, 30 Nov 2009 19:09:00 +0000

No, we didn't fall off the face of the Earth.... We have been pretty busy though! Sonya had her cast adjusted last week, since it was compromising her femur! She now has a pretty festive short leg cast! She has been able to maneuver much better in this! She can now crawl in a better position as well as stand without having the cast dig into her thigh putting unnecessary pressure on her femur! Here she is feeding her monkey some Elmo crackers! Ethan: this monkey has been attatched to Sonya ever since she broke her leg! He's her good buddy now!

We had a wonderful Thanksgiving and spent our night with Ian's family. Great food and wine, good chats and laughs, and smiles all around! The girls loved playing with all of their cousins! I can't believe how much our family has grown in the past few years! We are so thankful to have them all in our lives!

We visited my sister last week also and attempted to get a good photo of all the girls together as well as family portraits for my sister's family. Taylor was a little fussy in the beginning and by the time she was all calmed down, Sonya started her crankiness. We did manage to get this photo, not too bad... although Taylor is crying.... and I managed to get a few family portraits done for Meghan, Shay and Taylor.

Can you believe I went out with all the crazies and did Black Friday shopping for my 3rd year now?! I can't either! Well, I did... I braved Toys'r'us at 12 midnight, waited in line for only 30 minutes to get in, but once I was in it was a quick push and shove fest and all ended with an hour and a half wait in line that actually zig zagged through every one of the isles in the store! It was like Manhattan traffic inside a cramped store, but worse! Then it was on to Target at 4am after a quick coffee stop. I waited in line for 2 hours outside in the freezing cold with a t-shirt and sweater (forgot my coat, grrrr...). Turns out that Target didn't open at 4am, they opened at 5am! @#$%! The stupid Black Friday ad had a typo! Everyone was thoroughly pissed! We all bum rushed the store, people were going absolutely nuts, grabbing big plasma screen TVs, dual portable DVD players, Wii consoles and games...... The electronics department was flooded with desperate shoppers looking for their bulls eyes! However, the big ticket items were actually hid throughout the store in odd locations! DVD players were in infants/toddlers clothing and plasma TVs were in intimate apparel! Wow, what exactly is that implying? "Not now honey, I want to watch the game on our new big screen plasma!" Hahaha! As chaotic and crazy as it sounds, I still had some fun! The bummer of it all this year was that most of the stuff my kids wanted was not on sale! I managed to get a few good deals, but nothing really worth the wait and aggravating drivers out there on the road that day. I didn't get the item of the year that Akela wants. Zhu zhu pets, anybody?! These things are completely stupid, but she really wants one. I have a rain check but there's a good chance that hell will freeze over before they have them in stock before Christmas. Ah, oh well! There's always her January birthday! At least she forgot to ask Santa!

Here are the girls all ready to meet Santa! We were 3rd in line but ended up waiting for a ridiculous 40 minutes to see good ol' St. Nick yesterday because they were having technical difficulties with their cameras and they wouldn't let us use our own nor would they let us just see Santa anyway! FRUSTRATING!!! The girls hung in there though without any significant meltdowns!

The huge tree at the mall! Each of the toy soldiers, drumming bears, dancing dolls, jack in the boxes and ornaments were automated! They all danced in beat with the Christmas tunes that were playing. The tree truely sparkled and delighted all of the kids! This was the only thing that kept Sonya somewhat entertained and sort of calm while we waited!

Completely terrified by the big red jolly dude, Sonya clung to me like tight pantyhose, and that's how I ended up in this photo. Ignore me... and try to see past Sonya's scared stiff face. Yay Santa!

In other news, Sonya has been doing great with her physical therapy appointments! She loves Anne who comes to our house "to play". I'm so glad that she believes that she is strictly playing and not doing any work! Today she was a bit stubborn when we went to go see Sue at CHOP for PT. She didn't want to stand up to put the toys in the bucket. Only a few minutes later she was ready to walk with the pediatric walkers that Sue brought into the room for her to test out! Talk about a quick change of heart! We brought home a gait trainer, a loaner, to see how she does with it this week!

Sonya in the Rifton gait trainer with the center seat and back support. She's pretty angry about the back support in this photo!

Sonya with the center seat support and without the back... a little worried.

"My test drive gait trainer!"

She is a little tired in this photo and was doing a lot of leaning, but she can stand upright fairly well without the seat and back support. She is a little more hesitant about it, but she does ok with lots of encouragement!

She took 3 steps! I'm so excited to see her walking full force with this soon! She walked about 10 plus steps with the seat support at her PT appointment!

"Ok, I'm done! Time to kneel and sit!"

We will be checking into accessible parking as well as a power wheelchair very soon. This will ensure Sonya's independence when she is unable to walk because of a fracture or when she is in too much pain. This will also provide her much needed safety for when she enters preschool (in just a little over a years time) and the kids are running all about and likely to knock her over. It ought to be interesting teaching this very stubborn little girl how to maneuver the chair and not run over the cat!

Ok, better stop now... There's more to come soon, if only I could slow down the frenzy of my insanely busy schedule!

Meeting Taylor!

noreply@blogger.com (Melissa Swartley) — Sun, 22 Nov 2009 21:52:00 +0000

Since we have all had some nasty colds recently including the Swine Flu, we kept away from the newest member of our family, Taylor. Finally the girls and I are all feeling well with no residual effects from our illnesses, so we went to visit my sister Meghan and her first born sweetie Taylor! The older girls enjoyed meeting their newest cousin! Sonya and I visited once before, so I think she was not as impressed as she was the first time.

All four pretty girls together!

Akela and Zoe with Taylor

Akela : "I can't hold her 'cause she's too heavy"

Taylor making a face at Akela's remark.

Don't touch me "Tater!"

Sonya's thoughts : "Just because you're the newest cutie here, doesn't mean I'm going to let you have all the attention!"

Wow! I can't believe Taylor is going to be one month old already! (Dec. 1st)

On another note, Sonya is doing really good! I'm a little worried about her leg cast not going up high enough. It comes to an end right where she fractured her femur the last time and I worry about too much pressure being put on that area. It's just not safe looking. So, I'm calling her ortho tomorrow to see if we can come in and have it either extended higher or cut down lower below the knee. I'll update soon! Here's my monkey standing up! She has been climbing and all!

Friends and Boo Boo's!

noreply@blogger.com (Melissa Swartley) — Thu, 19 Nov 2009 19:30:00 +0000

Yesterday we got to meet the Weisser family!!! Jenn, her husband Fred, Ethan and Collin met up with us in Quakertown (our town) at the Chick-fil-a restaurant as they were on their way down from NY to go to duPont for Ethan's annual check up!

We had a great time chatting and getting to know each other a bit better and the kids had an absolute blast playing in the indoor activity equipment. Sonya loved climbing up and chasing the boys through the tubes! Both Ethan and Collin are even cuter in person! Ethan has such a wonderful imagination! He yelled down to me from above, sitting in the car shaped cubicle of the play set "Hey Melissa! I have a flat tire!" He loved it when I pretended to blow up his tires! He has such a great personality and I can see Collin is going to be following in his brother's footsteps too! We all had a fantastic time!

Unfortunately Sonya sustained another boo boo towards the end of our get together. She has really been pressing for her independence and I have been gradually letting go, scared to death about it of course, but I have to let her live it up and learn her limitations.

Sonya decided to go down the twisty slide without me for a change. She gave me a great big scream of a "NO!" as I tried to get her to "Go with mommy!" and she turned around, feet first and took the plunge. Unfortunately, I didn't think to remove her rubber soled shoes before I let her start playing in the play set so on the way down the slide her foot gripped the slide, tripping her up. Her leg twisted up behind her and underneath her bum causing her left tibia and fibula (lower leg bones) to snap and break just above her ankle. For you Eagles fans we call it the "Terrel Owens break".

Thank you sooooo much to Fred!!! Fred was awesome and went out to my car to get Sonya's break kit, brought it in and then helped me by holding Sonya's leg while I splinted it. If you ask me, this is no small feat to hold a child's broken leg after you just ate lunch! Thank you Fred!!!

We said our goodbyes to the Weisser's and headed to the local ER to have Sonya xrayed and to get her splinted with cast material. She broke lower on her leg than I expected. Both bones snapped below her bowing. I could have sworn I felt a break at the bow where she is currently 70 degrees, but the xrays show nothing in that area. After a chat with duPont, we were told to go home with Sonya splinted and make our way over to duPont in the morning. This is typical with this kind of break. Had this been her femur again we would have gone straight to duPont for surgery and heavy pain control (morphine and lorazepam). With this kind of fracture we home medicate with Tylenol with Codeine, Motrin and Diazepam (for the spasms) and we wait for the swelling to go down before casting the following day.

Sonya did ok over the night. She had a few (about 5-6) bad spasms that woke her out of a sound sleep, but it was nothing like her femur break spasms that woke her every 5 minutes the whole night and then continued after surgery for a few days. She seems to get pains about an hour before she is due for meds, but 15 minutes after she receives her meds she is LOOPY! Hopefully we won't have to medicate for too much longer and she will start to feel better!

Sonya is sporting a pretty green long leg cast! Thank God she is not in a spica again! I was really worried about that! We are thinking of painting red and white striped candy canes on it for Christmas! She will be in the cast for 4-6 weeks and we will be following up in 3 weeks to see where she is in the healing process. We have to be really careful with her when she decides she is ready to stand up in her cast because of where the cast comes to an end. Her cast ends where her femur snapped back in June. She will be more susceptible to fracturing that same spot because of the casts edge being right there. If she sits down funny, looses her balance while standing up in the cast and falls or gets her leg cast caught in an awkward position we could be heading back into the hospital with an even worse situation. So guess who is going to be hovering?! I don't expect her to stand up in the casts until she is at least 3-4 weeks into healing. She might not attempt it at all, but she is known as an OI dare devil! Time will tell!

I feel bad that Ethan had to witness the sliding board accident! He really didn't like to go down slides before the accident, now he really isn't going to want to go down! Don't worry Ethan! Sonya is doing good! Well that was our adventure for the past two days! I couldn't think of breaking a leg in any better way! Sonya was having a blast and living it up, just the way she should be living her life!

Here are the pic's! This bunch was on the move constantly, it was tough getting a photo of them all together!

Sonya and Ethan!

Sonya climbing through the tubes!

Sonya chasing cutie Collin!

Three little monkeys!

Coming out of the car shaped part.

Resting for a very brief moment!

Ethan being very cute!

Collin leading the way!

Hanging out in the car! I love Ethan's shirt! That's right Ethan, your mom does rock!

Looking for the slide!

Aha! I got all 3 of them in the picture!

Ethan showed me his special wrist bands to keep him from getting car sick!

What a great time!

Sonya in the ER with the cute monkey the Weisser's gave her! She cuddled with monkey the whole time!

Sonya's 16th and 17th fractures.

Feeling comfortable in her pretty green cast, men's tube sock on the other leg and chillin' on her beanbag! The boxers are a size 4 and huge! I had them from her spica days... I have to find some shorts that fit around the waist now but go over her cast without awkward positioning.... this should be interesting considering the racks are full of winter clothes! I think I'll be altering!

Green for the holidays!

Advent Paper Cake

noreply@blogger.com (Melissa Swartley) — Tue, 17 Nov 2009 20:42:00 +0000

Check it out over at my crafting blog Testing 123!

Swine, Conferences and Friends

noreply@blogger.com (Melissa Swartley) — Mon, 16 Nov 2009 00:24:00 +0000

So, our week had a pretty crumby start, but things are much better now! Sonya and I came down with the flu Monday night. I'm 99.9% sure that it was the swine and it was just absolutely awful! Sonya's fever peaked at 101 and was quickly under control with Motrin. Unfortunately for me I was pretty much hallucinating after 6 hours of enduring a fever of 103.5. I just started seeing a bunch of yellow dots over everything, no pink elephants flying around, but still NOT fun! We both had headaches from hell. Sonya held her head continuously and cried "boo boo" frequently, while my head pounded so bad that it hurt to keep my eyes open and I requested that all the lights be kept off in the house even the ones that I couldn't see from my view on the couch. We both barely ate, we had no appetite whatsoever and I tossed my cookies twice. I'm not even going to tell you the other end of it, I'm sure you can imagine the worst ever. We pretty much slept on the couch all day for 3 days straight. Blah!!! The older girls and Ian managed to escape the "on death's door step illness" and Sonya and I started feeling better on Thursday which was perfect timing since I had a parent teacher conference with Zoe's teacher.

Both Zoe and Akela are doing very good in school! Akela is making good friends and is a very good listener and rule follower! I am very proud to report that Zoe is above average in many different aspects of her education! She has been doing a phenomenal job with reading and comprehension and is very thorough in revising her finished work! Zoe is also a great speller! She impressed me just a few weeks ago with "Australia" and "Atlantic Ocean". They are currently learning about different geological formations. I always worry about how my older girls are doing both academically, socially and mentally because I find that I don't spend as much time with them as I do with Sonya. Taking care of a physical special needs child is very time consuming and exhausting to say the least. Hearing how great my girls are doing in school was an immense relief and warm happy moment! It feels great to know that I am still doing good with them too!

Sonya got to meet her new cousin Taylor this week! We planned on having all the girls meet their cousin sooner but darn that SWINE! So when Sonya and I were finally feeling better we went for a visit with my mom. The older girls were spending the weekend with their Nanny (Ian's mom) so we will have to fit in another fun visit real soon so that they can meet Taylor as well! Sonya was quite interested in Taylor at first and helped comb her hair. We switched to just the soft brush though since she was a little rough with the comb. She also liked covering Taylor up with the blanket. That was pretty much it, then Sonya wanted to show off her climbing skills and started monkeying around the couch and then cruised my sisters coffee table. Sorry, no pic's at this time... forgot my camera... Meghan can you send me some?! Here's this pic from last visit though! Taylor is so stinkin' cute! She is wearing one of my girls outfits!

So, the last time I blogged I had mentioned that there was another little girl with OI living just minutes away from us and we were excitedly hoping for contact with her family. I'm happy to say that we met!!!! Sonya has a new friend named Evelyn who just turned 2 years old and also has OI! Evelyn also gets her care at duPont and sees the same doctors that Sonya has. We heard of her through Dr. Bober and his assistant Lauren and just couldn't believe that they were only 10-15 minutes north of us in a town that I visit frequently! Small world (pun intended)! Evelyn is a little more severe with her OI than Sonya, but not by much! She has had 20+ fractures and Sonya has had 15/16 (not sure about her newest possible). Evelyn is just learning to stand and she is in love with Elmo! Hmmmm... does this sound familiar?! Sonya and Evelyn are like twins... two peas in a pod! We had Evelyn, her mom Sandy and her dad Bob over for a visit today and it was just excellent!!!! Sonya and Evelyn played together very nicely and even hugged each other a few times! They passed the soft bouncy ball back and forth, played a bit of dress up, checked out Sonya's flash cards and of course Mr. Potato Head. It is so wonderful that Sonya now has a friend who understands how to play very gentle with her as well as how it feels when there is a break. I hope the two of them become very close good friends! We plan on getting together again real soon! Too bad that we are on different schedules for PAM. It would be awesome for them to sit together during their treatments! Eventually maybe that will work out! Sorry, no pictures of the cuties playing together, I was enjoying the moment too much! Next time!

I do have this series of photos though... Enjoy!

Akela: "No Sonya, this is my castle!"

Sonya: "But, I love you Akela!"

Akela: "Stop loving on me Sonya!!!!"

Sonya: "But, I LOVE YOU SOOOO MUCH Akela!"

Akela: "OOooowwwww! Sonyaaaaa!!!!"

Akela : "Waaaaaaaa!!! Sonya, you made me break my castle!!!!"

Me thinking: Those pictures clearly depict no castle destruction.....

Sonya: "Waaaaaaaa!!!! I don't know why we're screaming, but Waaaaaaa!!!!"

Missing Treatment

noreply@blogger.com (Melissa Swartley) — Tue, 03 Nov 2009 23:18:00 +0000

Well, it turns out that Sonya will NOT be getting her infusion this week. The last time we went for her PAM infusion it was absolutely awful! As I'm sure you all remember, it took hours to get her IV placed and she had 3 blown out veins along the way. Well, apparently she was overdosed the last time as well and that is why she can not receive an infusion now. Sonya receives a PAM infusion every 8 weeks and receives the 8 week dose. Last visit she was given the 16 week dose, double the amount of what she should have received! I don't know how this all happened and our doctors apologized profusely. As of now Sonya is not showing any ill side effects from this overdose (extra fracturing, reactions) and most likely she will be just fine given that the infusion amounts are measured over a years time... so there is room for correction. We will be going back in 5 weeks for her next infusion and her doctors will modify her dose amount to balance out the yearly dose. Her doctors do not think that she will make it another 8 weeks without another infusion as her bone turnover is faster than that of a 4 year old (who typically receives the 16 week infusion). Her total PAM for the year will be more than what she is supposed to get at this age regardless of the modifications to the amount that she receives at future appointments. This can be troublesome because too much PAM can cause the bones to become hyper dense (more brittle) causing them to break more often, but we don't feel that she has been overdosed to that extent. Sonya will be more vulnerable to fracture during the next 5 weeks however because nobody knows for certain how fast her bone turnover is and how fast the drug will run out of her system. Bummer that all of this happened, because before we found out of her overdose, the IV team placed her IV on the first try, fairly easily and with no complications. Uggghhhh!!!! Well, at least we know that the port surgery isn't as likely to take place just yet!

On top of finding out that the IV issue is not a huge problem right now, two more good things came out of this appointment! I talked to her doctors about not being able to get a hold of our PT recently and they have told me that they would handle it and try to set up an appointment ASAP for us so that Sonya can get her bilateral clam shell braces for her lower legs and her pediatric walker! She has been cruising furniture non-stop and climbing everything in sight! The braces will give her more support on her 70 degree bowed tibias and the walker will get her more interested in walking all about while providing the necessary support! I can't wait to see her in motion! She has also been attempting to stand up in the middle of the room, but so far just keeps falling down.... very scary for me to watch, but at the same time I have to let her go. She'll never fully enjoy life if I hover over her like a crazed, worried, overprotective mom. Then we were told that there is a little girl named Evelyn who is 2 years old and has OI and guess where she lives??? Seriously, 10 minutes away from us!!!! The doctors are going to try to contact her family to see if they would like to have contact with us! Maybe Sonya will have a playmate who understands how to play gently! I'm so excited! I hope it works out!

Here is that picture I promised you all of Sonya content with her pumpkins new look! She thought he was cute with his smile!

In other news.... I became an Aunt again, but for the first time on my side of the family!!!! Taylor Elizabeth Hutton was born on November 1st 2009 at 3:26pm, weighing in at 9 lbs and 9 oz and measuring 22 1/2 inches long!

She is absolutely beautiful and perfect in every way! Congratulations to my sister Meghan and my brother-in-law Shay!!!!!

So sweet!!!!

Coming home!

Happy Halloween!!!

noreply@blogger.com (Melissa Swartley) — Sat, 31 Oct 2009 17:26:00 +0000

HAPPY HALLOWEEN EVERYONE!!!

Here's a little.... ummmm ok a big recap of what we have been up too! I'll do it in pictures, less words... enjoy!!!

Snow White Sonya and her poison apple!

Belle Beauty Zoe!

Ariel Little Mermaid Akela!

"She's got legs! Human legs!!!"

My 3 pretty princesses!

Carving time!

Akela checking out the insides of her pumpkin!

Zoe and her pumpkin!

"Ewwww!!! Guts!!!"

Separating the guts and seeds.

I was not expecting this reaction from Sonya... She was really upset about her pumpkins insides.

You have got to be kidding me! That's what is inside of my cute pumpkin??!!

Oh the horror, THE HORRORRRRR!!!!

Daddy please put it BACKKKK!!!!

That's just awful!!!

Don't worry, she eventually got over it and dug in!

Daddy and the girls gutting the pumpkins so mommy can carve them.

Zoe and Akela admiring mommy's carvings. Sonya was so tired, she went to bed and missed the results... will post more pictures after trick or treat tonight, hopefully I'll get a good one of her with the lit up jack-o-lanterns!

HAPPY HALLOWEEN EVERYONE!!!

"Building Self-esteem in Children / Teens with OI"

noreply@blogger.com (Melissa Swartley) — Wed, 28 Oct 2009 19:27:00 +0000

I wanted to share with you all a webcast that I watched last night "Building Self-esteem in Children/Teens with OI". The webcast has been saved and can be seen at the link below. It is approximately an hour long discussion given by Kara Ayers whom herself has type 5 OI. Kara is very informative on the topic and also has her own website where she discusses the broad spectrum of disability issues. Currently Kara is pursuing her PhD in Clinical Psychology with an emphasis on children with chronic illness and disabilities, specifically children with OI. She is finishing her dissertation on the psycho-social effects of growing up with OI. For the POLP reading this post, some of these topics can be very helpful for you and your LP as well! Not all of this discussion is necessarily directed to just children and adolescence living with OI, but can be applied to many other children with varying abilities who spend a considerable amount of time around hospitals and whom have many different appointments with varying specialists frequently.

In Kara's webcast she discussed some helpful children's books. My mom is planning on getting these books for Sonya for Christmas. I will post a review on these books with my thoughts in a future post!

Kara's Website : http://www.karaayers.com

Link to Webcast : http://www.ustream.tv/channel/building-self-esteem-in-kids-with-oi

Enjoy!!!

The Gummy Bear Song - Long English Version

noreply@blogger.com (Melissa Swartley) — Tue, 27 Oct 2009 19:39:00 +0000

Warning: This video will have your kids hooked! Sonya is absolutely in love with this song! I have it stuck in my head since she requests viewing it on youtube at least 5 times a day! I have to tell her that he went night night!

Black Apple Doll

noreply@blogger.com (Melissa Swartley) — Mon, 26 Oct 2009 17:17:00 +0000

I made this cute doll for Sonya last night out of some scrap fabric in just under an hour and presented it to her in the morning when she woke up! She loves her new dolly! For more info on where to find the free tutorial and pattern head over to my craft blog at Testing 123! If you like, I have a button to add to your blog!

Sonya making her new dolly dance!

Giving dolly a kiss!

Checking out her scarf!

Of course she had to taste her button too! Don't worry it's sewed on super tight!

Dolly

"Dolly" hanging out with "bun-bun"(bunny), "rrrarrr" (lion) and "pairy how" (fairy house).

Napping.... ZZzzzzz....

I think I sewed her arms on a little funny. She looks like she is in a permanent ballet position! I'll have to give this tutorial another shot and make dolly a friend or two! Now my older girls want one too!

Pumpkin Patch

noreply@blogger.com (Melissa Swartley) — Fri, 16 Oct 2009 05:26:00 +0000

I took the girls over to AGA Farms in Bedminster this past Monday to pick some pretty pumpkins in the patch, run through the corn field maze, check out the cool emu and of course snap some beautiful pictures of my cute gals having a blast!

Well, it seems the pumpkin farmers had a rough season here in PA. There wasn't much to choose from, in fact most of the pumpkins were rotting already! We searched for about 40 minutes and managed to snatch up 3 decent sized good lookers!

Telling pumpkin stories!

"So Mr. Pumpkin....."

Akela is such a ham for the camera these days!

My pretty Zoe.

All on board the pumpkin wagon!

Zoe and Akela did a great job of hauling the wagon and loading it with the pumpkins while I carried Sonya. Boy, I can't wait for her to walk!

Zoe found the first good looker!

The girls got to see a personal air plane take off on the farm's land! Sorry, no pic's of the plane, I was too busy getting this cute shot!

Where's Sonya? There she is, she's the cute pumpkin in the middle!

"Yay punkins" is what Sonya saying in this photo! I love how she says pumpkins!

Checking out our pumpkins!

Goofballs!

AGA Farms

The farm's lonely emu!

Sonya was completely petrified and screamed the second I even came remotely close to the big bird!

Funny, the older girls were also pretty intimidated by him too! Akela was a little more on the brave side though compared to Zoe.

Getting to the edge of the cornfield maze! Look at that ominous looking sky!

In the thick of the maze! Storm a brewin'!

Akela and Zoe call this picture "taking a corn field shower!" I don't know, I think they were being a bit goofy!

Sonya wasn't sure what to make of it!

She did enjoy watching her sisters being completely silly though!

That was fun!

Bonus : We managed to get outta there before the down pour of heavy rains!

The Second Opinion

noreply@blogger.com (Melissa Swartley) — Wed, 14 Oct 2009 23:50:00 +0000

Hi everyone!

We went down to Kennedy Krieger Institute's OI Clinic in Baltimore Maryland today to see Dr. Leet. She looked over Sonya's x-rays and said that given the fact that Sonya has not fractured frequently in her legs since birth and her only having a few PAM treatments, she is not ready to be rodded. She said that she definitely is a good candidate for rodding as her femurs and tibias are very bowed, but she needs to have more PAM first! So we are waiting. She also cleared up the C1 C2 instability debate. Dr. Kruse is saying there is NO issue while Dr. Ain feels he sees something there. Dr. Leet says that when Sonya is old enough to understand how to move her head from chin resting on chest to eyes gazing at the sky positions in a smooth transition and do so on command then they could perform a CT scan and get a better idea of what is going on there. We are all hoping it is not a Basilar Invagination issue. So, guess who's going to start practicing this smooth transition of positions?!

In the meantime Dr. Leet feels that Sonya should be getting Physical Therapy 2-3 times a week plus swimming time. She also says that Sonya is ready for a pediatric walker and that she could use some clam shell AFO's for bracing her legs. We will be calling our Physical Therapist over at duPont tomorrow to get the ball rolling on all of this! I'm so excited for the walker! Sonya has been starting to cruise (taking 3 or 4 steps) furniture and has been pulling up to stand at even higher pieces of furniture in our home.

We will be having Early Intervention coming out to our home on the 19th of November for an evaluation so hopefully we can get the 2-3 days a week of Physical Therapy through them and that would mean no traveling for us!

I am happy that we sought out a second opinion on rodding surgery. I was disappointed that our orthopaedic surgeon at duPont wasn't even willing to discuss rodding with me until Sonya was 3 years old. Now that I know the reasoning behind his not wanting to rod her until later, (Sonya's bones are just not strong enough to take on that surgery yet) I feel better. Thank you everyone for your well wishes on this trip!!!

I know you all need your Sonya fix! So here she is in all of her climbing and standing glory!

Mommy's Arms

noreply@blogger.com (Melissa Swartley) — Tue, 13 Oct 2009 04:55:00 +0000

Yes, one of these days Sonya is not going to fall asleep in my lap anymore. It may still be awhile off in the future, but probably not too far. She's getting closer to being 2 years old! Unbelievable! I'm going to miss these days!

Dwarfism Awareness Month!

noreply@blogger.com (Melissa Swartley) — Wed, 07 Oct 2009 22:07:00 +0000

When we first learned that Sonya would be born a little person I was surprisingly at peace with that fact. I knew very little about dwarfism at the time, but I knew she would be ok being little. My only concerns for her were purely medical. We were told that she had one of two lethal types of dwarfism. Lethal because she would have respiratory issues once born. All I could think about was "Please God let her be able to breathe and live!" I knew that there would be struggles when it came to her stature, but that she could overcome those struggles and live a happy life. I just prayed that she would get to live that life! To read more about our begining click on Sonya's Story.

I can't tell you how many times I have had strangers, who have just learned her age, tell me "Oh wow! She is really tiny! Was she premature?" After my response of "No, she is a little person." they look at me like I have two heads, like they are thinking "isn't that what I just said?" It amazes me how many people do not recognize the term "little person"! I literally have to spell it out for them. "A type of dwarfism." Even still, most people just don't get it. This of course most often ensues in a 30 to 45 minute conversation about how many types of dwarfism there are, as most people who have heard of dwarfism are only familiar with Achondroplasia, and of course the lengthy discussion of Sonya's particular type of dwarfism, Osteogenesis Imperfecta type 3.

Most people with the many different types of dwarfism would not consider their type of dwarfism as a disability. For Sonya it is different. "Frequent fractures of the long bones, the tension of muscle on soft bone, and the disruption of the growth plates lead to bowing and progressive malformation." is stated by the Osteogenesis Imperfecta Foundation. These frequent fractures hinder her ability to be as mobile as a person who is of average stature and unaffected by OI. Sonya doesn't let any of this "hindering" get in her way! She is a very determined little girl and nothing will stop her from achieving great feats! She dragged around a 5 pound half body cast at just 17 months of age (her stats : 17 pounds and 27 inches at that time) moving from room to room with a broken femur for 7 weeks! She found a new way of getting around after she broke her arm at 14 months of age and could no longer bear pressure on it to crawl! Sonya amazes us and others with her strength as she pulls up to stand on her 90 degree angulated weak and brittle femurs (thigh bones) and again when she hoists her small 27 inch frame up on top of our couch that is almost as tall as her at 20 inches. She is strong despite her fragile bones and small body. It may take her longer than the average height toddler to accomplish things such as walking and running but she'll get there when she's ready! She is one amazing sweet girl and we are blessed to have her as our daughter!

Aside from OI and dwarfism being a very small part of who Sonya is, she is a typical almost 2 year old! Sonya loves hamming it up for the camera, giving a little bit of an attitude here and there, telling us "NO!", playing dress up and tea party. She loves building with her blocks, naming the pictures on her flash cards, looking at her books and playing with her baby dolls. Her appetite is small like her and she is generally a picky eater as are most toddlers. She could watch Elmo all day if I let her and she finds it amusing when the cat meows loudly after she pulls his tail. She enjoys taking rides around the block in her Step 2 Car Buggy and her Radio Flyer Wagon when she is in a cast. She is terrified of the lawn mower and the vacuum and immediately wants to be held and comforted when either are running. Sonya is completely all out obsessed with Beyonce's Single Ladies hit song and requests frequent plays of the video on YouTube. One of these days I will have to catch her on video dancing and singing to it! She hates being contained, in the highchair, car seat, stroller, or by a gate just like any other toddler usually does and will do anything in her power (usually screaming at the top of her lungs and throwing a full out tantrum) to be freed! Surprisingly she is just awesome at going to bed for me! She almost always, with only a few rare exceptions, falls asleep within only 5 minutes after I put her to bed!

Sonya makes my heart skip a beat every time she says "I ya you" (I love you) and she has me laughing in stitches when she says "Jus poopie!" (Just poopie!) referring to whats in her diaper at any given moment. She gives warm squeezey hugs and slobbery kisses and you can bet if you are with her for just 10 minutes you will see her pick her nose at least once! She will pout when you tell her to stop. Sonya loves to get her hands on my phone, my keys and everything else that she can't have that lives inside my purse. She can be silly and quirky at times but she is always just super cute, sweet and lovable!

As you have read, Sonya is your typical almost 2 year old. Being a little person whose bones are fragile and break easily is just a small part of who Sonya is. Although her type of dwarfism, OI, is a serious condition and it does affect her daily, she doesn't let it own her or run her life. When it comes to Sonya life, she is the star and she runs the show!

Sonya with her two older sisters Zoe and Akela last October (2008)

Sonya this October (2009) wearing the same exact outfit, same size!

Sonya pulling up to stand October 2009

Sonya with her Daddy's size 10 1/2 work shoe October 2009

Ok, so now I'll take a moment to express my feelings about the "M" word. It is flat out unacceptable and offensive. Many people compare it to the "N" word that is used with hate and ignorance to describe African Americans. Both of these terms should be eliminated from our language! I also think of it this way : using the "M" word to ridicule someone of short stature is just as terrible as it would be to point and laugh at people with other genetic conditions. Is it acceptable to poke fun at someone with Alzheimer's Disease, Autism, Cerebral Palsy, Diabetes, Downs Syndrome, Epilepsy, Hemophilia, Muscular Dystrophy, Parkinson's Disease or Spina Bifida....etc... The answer is NO! So why do people think that it is acceptable to ridicule, point and laugh or whisper about people with a type of dwarfism? It's a genetic condition! It comes down to this : We are ALL human! We all come in different shapes and sizes, colors and personalities. Isn't this something that is taught to us all when we are in preschool or kindergarten?! Shouldn't it have sunk in by now?! Ok, I'm off my soap box.

Now I pass on this message from Little People Of America. Here's to spreading dwarfism awareness!

FOR IMMEDIATE RELEASE CONTACT:
October 1, 2009 Gary Arnold
Little People of America
1-714-368-3689
GArnold@accessliving.org
LITTLE PEOPLE OF AMERICA RECOGNIZES OCTOBER AS
NATIONAL DWARFISM AWARENESS MONTH

October has been declared National Dwarfism Awareness Month by Little People of America. Little People of America (LPA) is a national support organization for people with dwarfism and their families. LPA provides peer, parent and medical support for the dwarfism community.

LPA began in 1957 with actor Billy Barty and 20 short-statured friends. The first LPA meeting was monumental for a group of people who were severely mislabeled and misunderstood by society. Today, Little People of America is a thriving international organization providing much needed support, education, educational scholarships, and community outreach.

In declaring Dwarfism Awareness Month, LPA hopes to raise positive awareness around dwarfism, address common misconceptions, and increase opportunities for people with dwarfism around the country.

“People with dwarfism are no different than any other person. We may just need a well-placed stool. Our members are children, college students, business professionals, doctors, engineers, mechanics, artists and teachers. We can do anything an average-height person can do,” says Lois Lamb, LPA President and a person with dwarfism.

“Raising a child with dwarfism is new for most parents. Being part of LPA has made a tremendous difference. My daughter and I both have great friends and role models. High school is not an easy time for most teens, but neither of us feels alone in dealing with any extra challenges,” says Joanna Campbell, average-height mother of a teen with dwarfism.

More about LPA and dwarfism:

* There are over 200 distinct forms of dwarfism and skeletal dysplasias.

* People with dwarfism are generally not taller than 4′ 10″ at adult height. The typical height range is 2′8″ to 4′5″.

* Eighty percent of people with dwarfism have average-height parents and siblings.

* There are an estimated 30,000 people in the United States and 651,000 internationally with a type of dwarfism.

* In July 2009 the word “midget” was declared inappropriate and offensive. Preferable terms are: having dwarfism, short stature, little person, lp, and the medical terminology use of dwarf. A person’s name is always the most preferred.

* LPA has registered a complaint with the FCC over the use of the “m” word. Our goal is to raise awareness around the offensive impact of the word in order to eliminate use of it in media, popular culture and in everyday language.

* LPA has 6500 members across the United States and includes 70 chapters active in all 50 states.

* Skeletal Dysplasias affect bone growth, but generally do not affect cognitive abilities.

* LPA hosts an annual national conference each July which draws 2500 attendees for a week of activities, including educational and medical workshops, sports, and social networking and events.

For more information about National Dwarfism Awareness Month, please visit www.lpaonline.org or call 1-888-LPA-2001. Events supporting National Dwarfism Awareness Month will be occurring throughout the United States all during the month. Contact the chapter in your area, or the Regional Coordinator for Dwarfism Awareness Month listed on the website.

The National Dwarfism Awareness Month program is dedicated to increasing public knowledge about people with dwarfism.

Mission of LPA “LPA is dedicated to improving the quality of life for people with dwarfism throughout their lives while celebrating with great pride Little People’s contribution to social diversity. LPA strives to bring solutions and global awareness to the prominent issues affecting individuals of short stature and their families.”

The LPA National Office is located in Southern California. You may also contact Joanna Campbell, Executive Director at info@lpaonline.org or (714)368-3689.

Zoe's First Bento!

noreply@blogger.com (Melissa Swartley) — Wed, 07 Oct 2009 01:10:00 +0000

What the heck is a Bento, do you say? Check out Wendolonia! I ran across her blog when I was searching for some lunch box ideas for Zoe. I am sooooo tired of packing the same old thing for her when she doesn't want to buy the "too weird" lunch cafeteria food or when we are strapped for cash. These days it's mostly our bank account that is screaming no to buying a lunch. So I have been trying to save us some bucks by getting Zoe to pack more often. Anyways, Wendolonia has some awesome ideas for packing a cool lunch!

Bento is a single-portion takeout or home-packed meal common in Japanese cuisine. A traditional bento consists of rice, fish or meat, and one or more pickled or cooked vegetables as a side dish. Haha, my kid would probably faint at the sight of that lunch! We had our own take on the bento! Containers range from disposable mass produced to hand crafted lacquerware. We used some airtight lock and lock containers and some pretty colorful silicone baking cups for ours! Although bento is readily available in many places throughout Japan, including convenience stores, bento shops, train stations, and department stores, it is still common for Japanese homemakers to spend considerable time and energy producing an appealing boxed lunch. Bento can be very elaborately arranged in a style called kyaraben. Contests are often held where bento arrangers compete for the most aesthetically pleasing arrangements. Kyaraben is typically decorated to look like people, animals, or characters and items such as flowers and plants. There are similar forms of boxed lunches in the Philippines (Baon), Korea (Dosirak), Taiwan (Biandang), and India (Tiffin). Everything is separated neatly in little compartments. I just thought it was a much better presentation than wrapping the sandwich up in saran or foil and throwing it along with a bag of chips into the lunch box.

Zoe's first bento!

I kept it real simple!

Red seedless grapes

Pirates Booty! AKA : cheddar puffs

PB&J finger sandwiches cut into pumpkin shapes with a cookie cutter. Sorry, no pictures of the pumpkins!

Cinnamon and sugar crisps!
The recipe = EASY!

Take a flour tortilla, spread a thin layer of butter over top, sprinkle with cinnamon and sugar mixture, slice in strips with a pizza wheel and bake in oven at 350 degrees for approximately 5-8 minutes. When the edges are slightly browning and the top is bubbling they are ready!

All together now!

For the record : She loved every bit of it!!!

Sonya Conquers The Couch!

noreply@blogger.com (Melissa Swartley) — Mon, 05 Oct 2009 03:22:00 +0000

Sonya, 20 months old, learns to climb the couch! Keep in mind Sonya is standing on femurs that are bent at 90 degree angles. She is only 27" tall and the couch seat is 20" high up! She has some very impressive upper body strength!

Our Story, Sonya's Story, My 100th Post!

noreply@blogger.com (Melissa Swartley) — Wed, 30 Sep 2009 01:21:00 +0000

I have been blogging for almost a whole year now and I can't believe that I never got around to posting "Our Story, Sonya's Story". Well, what better time to do it (besides the first post) than now on my 100th post! Geez, 100 posts! Where does the time go?!

This was quite hard for me to relive as I typed it, so hopefully the tears didn't obscure my thought process and this will come across as clearly as I intended it to be. It's a long story, but I didn't want to miss any of the details, as this is first and foremost a place where I document Sonya's precious life.

Ian and I were high school sweethearts and after 6 years of dating we tied the knot on June 22, 2002 only 8 months after our first daughter Zoe Elizabeth was born on September 14th 2001. Yes, Zoe was a little surprise, we like to do things a bit backwards! We lived with my parents until Ian finished college, I tried to finish as well and we both had steady jobs. In September 2003 we bought our first home! It was quite a fixer-upper but with the help of my dad, Ian's dad and my cousin Jim we had the place gutted, remodeled and cleaned up in 4 months time. New house equals new baby... Enter Akela Lillian! Akela was born on the warmest night here in PA on January 13th 2005 when it was an incredibly unseasonal 67 degrees! Shortly after Akela's birth Ian received the chance in a lifetime kind of job! Merck Incorporated finally gave him the call he had been waiting for after his application and resume was handed in years before! He gladly accepted and is still currently working there as a bio technician making some of the top vaccines that our children receive! I continued to waitress and bar tended at a busy restaurant. We were making great money, we had two wonderful children, life was good!

In May of 2007 we were happy to know that we were expecting a third baby! Ian and I always wanted to have 3 children and this little one would complete our family! The first 5 months of my pregnancy sailed by smoothly. I chose to have a level two ultrasound at my 20th week of pregnancy to compensate for the quad-screen blood test that I turned down. When I was pregnant with Akela my triple-screen (before there was a quad) had come back with a false positive for Downs Syndrome and we were under immense stress and worry for her until a level two ultrasound done at the end of my 8th month of pregnancy revealed everything to be just fine. At our 20 week ultrasound with our third baby our lives were turned upside down.

The ultrasound technician slid the transducer over my belly on the day before Zoe's 6th birthday, taking longer than the norm. I had a dress fitting to go to later that night for my sister who was getting married in a little over a month. My previous situation with Akela sent thoughts running through my head as I lay there trying to get the nerve to ask what was taking so long, what was wrong. Did I want to know, dare I ask? I had to press this tech's buttons and get an answer out of her or at least get her to go fetch me my doctor ASAP! She wouldn't tell me what she saw and ran off to go get my doctor. Those 10 minutes felt like an eternity, but nothing could compare to the next 18 weeks that I would spend worrying sick with the knowing of what would/could be.

The doctor came in and repeated the ultrasound again. He told me that my baby girl's femurs were extremely shortened and bowed and that he could see that her one foot was clubbed and that I would need to see some more doctors for another ultrasound. I couldn't believe what I was hearing "malformation, demineralized bones, dwarfism? lethal?" all unbearable, but "baby girl" sounded just wonderful!

5 days later after celebrating Zoe's 6th birthday and attending another party at Ian's brother's house over the weekend, I lay again on another cold hospital table while doctors slowly examined every inch of our baby girl's body through another ultrasound. I initially tried to think happy thoughts. Zoe was getting her picture taken at school that day, I pictured her pretty smile. I thought about Akela contently playing with her toys at home. The doctors kept finding more and more problematic bones. Now her tibia's were also shortened and bowed, her other foot was questionably clubbed as well and her chest was small and bell shaped, her respiratory health in jeopardy. The doctors then had me scheduled in 2 more days for amniocentesis and genetic counseling.

On September 20th 2007 we were told that our baby would not survive. She had one of two lethal skeletal dysplasias. Campomelic Dysplasia was most likely our baby girls' diagnosis with her bell shaped chest which would not support the growth of her ever expanding lungs. We were told that she would suffer respiratory failure shortly after birth. Osteogenesis Imperfecta (Brittle Bones Disease) type 2 did not sound any better. If our daughter was to have this dysplasia she would undoubtedly break almost every bone during her birth, ribs included which could potentially puncture her lungs. The trauma of that many broken bones during the birthing process would be enough to bring upon her death. The doctors painted our daughters future as dark as midnight and as cold as ice with no light or ray of hope to be seen. The amniocentesis I underwent gave no additional information, leaving us to wait and worry. I remember asking "If she has Osteogenesis Imperfecta, would having a C-section help?" My glimmer of hope was shot down with the answer "No, it will not matter which way you chose to deliver, your baby just will not survive, you should make the arrangements now for her funeral. I'm sorry." Just like that, just that cold.

I don't know exactly how much I cried over this devastating news, but I think it was enough to fill all 7 of our oceans. How could I possibly go about daily life with this knowledge of our future in my head at all times? What would I tell my older girls when mommy's belly became small again and there was no baby in her arms? How could I ever possibly live on after something so terribly traumatic? I prayed and prayed and prayed. I pleaded with God to let me keep her, even though I knew it was selfish. I just couldn't seem to get past just wanting to keep her.

I quit my waitressing and bartending job, I could barely face my boss to tell him why. I just came in for my shift the next day after the meeting with the genetic counselors, completely broke down in tears and told him why I had to leave and not come back until God knows when. He understood completely of course! I had follow up ultrasounds every 3 weeks to watch our daughters condition. At first those ultrasounds showed continual increase in significant bowing of her bones and then much later no changes at all.

Only 2 weeks after we were told that our daughter would not survive, my father was diagnosed with stage 4 lung cancer. In between my ultrasounds I attended and organized my sister's bachlorette party with the help of her wonderful bridesmaids as well as her bridal shower party again with her bridesmaids and my mother. I went through several dress fittings for her wedding as my belly kept growing and cried at home after every single one, wondering if there was any slim possibility that our baby would make it. I attended my sister's wedding and watched my father walk her down the isle, seeing him with his hair for the last time. I took my dad in for his chemotherapy treatments and brought him back home again seeing him continually get thinner and sicker. I took the older girls to see Bee Movie and went to Zoe's parent teacher conference meetings. I visited my girlfriend Danielle after she welcomed home her new baby boy Aidan, celebrated Thanksgiving and Christmas with our family and had my hair cut from it's long style into a short bob. After celebrating the new year we had Akela's 3rd birthday party a week early just in case our new baby girl planned on an early arrival. Somewhere in there I had a consultation for a C-section because I did not plan a funeral for our daughter, I wanted to plan a birth and I saw a C-section as hope, just in case her fate was Osteogenesis Imperfecta type 2.

Ian and me with Zoe and Akela (she really wants that beer!)

Our last Christmas with Dad

At 9pm on Wednesday January 16th 2008, 12 days before she was due and 7 days before my planned C-section date, my water broke on my vinyl covered new couch (just in case... good thing huh?!) and I started having contractions. After calling Ian's parents to the house to watch the older girls who were already tucked in bed and calling my doctor to notify him, we rushed off to the hospital to have an emergency C-section. We arrived at 10:45pm and I was in the surgery room and prepped by 11:05pm. At 11:20pm Sonya Collette Swartley entered the world screaming!!! I was so happy to hear her crying! Tears rolled down my face as I begged for them to let me see her! They slipped me a glance of her beautiful face for a brief moment before whisking her away to the neonatal intensive care unit. To me, weighing in at 8lbs 5 oz and 18 inches in length, Sonya did not seem small enough to qualify as a baby with dwarfism! Her apgar scores came back great too! She wasn't even put on oxygen! All of this I had been told from the nurses who frequently came in to give me pain medications through my IV as I waited impatiently to see, hold, feel, hug and kiss my baby girl! I don't remember how long it was before they finally brought her to me and Ian, but it was sometime around 4 or 5am I think. I couldn't believe that she was ok! We nursed and I held her so long, practically refusing to go to sleep because I just couldn't get enough of her.

Just a few hours old.

Our little Eskimo baby.

Sonya's club foot and severely bowed leg.

Here is where things get blurry. All family members came in to visit at various times. So many people came and I was pretty much hopped up on pain meds. My stomach hurt so much from the C-section and I felt like my whole front side was going to literally just tear right off and drop to the ground when I stood up for the first time. It was very uncomfortable to say the least. I don't think all C-sections are this painful, I just think that because of my extreme weight gain towards the end of my pregnancy and Sonya being my third child, those stomach muscles just were not what they used to be! I was sharing a room at the time with another mom who just gave birth to her second baby. Just as my mom and sister had come in to see us (for the first time or again, I'm not sure) a group of doctors rushed in and told them to leave, practically pushing them out and pulling the separation curtain in between me, my family and the other mom. My face fell as I held sweet Sonya in my arms. What was going on? There in the shared room they told me that there was something wrong (I gather from the numerous x-rays that they had taken of Sonya), that Sonya had Campomelic Dysplasia and that she didn't have long to live, that surely her lungs would be smothered by her too small rib cage and that she would need to have more testing done. "What?!!!" was all I could muster. "Do you want your own room?" they offered. I wanted my own damn room now! This was complete bull! Here I am balling, crying so hard I have probably terrified the mother on the other side of the curtain. My baby is going to die? Thoughts racing. Heart dropping. Sinking. Hands shaking uncontrollably. Couldn't they have at least offered me my own room before they killed all my hope?

The rest of my stay at that hospital is completely vague due to all of the trauma and drama that played out. All I really remember is praying and seeing how perfect Sonya was, her cheeks and lips pink with warmth, her dark thick curly hair so soft, her skin tone darker like Ian's (that little bit of beautiful Leni-Lenape Native American peeking through) and her long dark eyelashes occasionally opening up for me to see her gorgeous eyes looking right at me. Sure her foot looked different the way it was bent in half, toes kissing her heal and her leg curled awkwardly coming all the way up to her belly button and resting there naturally, not wanting to straighten, but couldn't those things be easily fixed if they needed to be?

We were transferred to the Children's Hospital of Philadelphia's NICU where we stayed for the next 4 days after the 3 days at our local hospital. X-rays, ultrasounds, blood tests, DNA tests, echo cardiograms and other tests were all run on her. She laid in a teeny tiny examination bed during our whole stay as Ian and I slept bedside in chairs next to her. We only occasionally left to grab something to eat. The cafeteria was all the way across on the other side of the hospital and all of that extra walking for me combined with some unavoidable stairs and no access to a shower caused an infection of my C-section incision. I could care less though and ignored it while Sonya was in the hospital. We met with Sonya's genetics team (yes a whole team just for her) and were told they were almost certain her diagnosis would be Campomelic Dysplasia, a dysplasia so rare that there were only approximately 80 cases in the world. They were 99% positive about their decision and sent out the first set of DNA samples to Cedars Sinai in California. We were told by all of her nurses in the NICU that she was the healthiest baby in their NICU despite her possible diagnosis, that she had no signs of respiratory distress or any other immediate issues and that she would be going home with us on the 23rd.

At the NICU at CHOP.

So many wires and monitors keeping track of her.

Zonked out after a DNA blood withdrawl.

Home sweet home! *Yawn!*

All three of my beautiful girls.

Teeny tiny hands.

Sonya with her teddy 1 month old.

We came home on the 23rd of January and picked up life where we left off. My C-section incision was clearly infected and 2 weeks worth of antibiotics finally cleared up the painful mess. We had some snow days, friends and family came to visit and Akela had her major follow up appointment with her cardiologist. The leaky valve and heart murmur had cleared itself up and one major worry was off of our shoulders! I continued to take my dad to his chemotherapy appointments. In March my dad went into the hospital and we found out that the cancer had spread to his liver. He came home on hospice on Easter Sunday and we all had Easter dinner together. We all visited my dad on the 29th and I took some pictures of him with all 3 of his grandchildren, my daughters. On the 1st of April dad lost his fight against lung cancer when Sonya was just 10 weeks old. He is missed so much and I wish he could see my girls today! Sonya has come so far!

My dad with all three of his grandchildren, my girls, one last time.

Sonya's first cast to correct her club foot.

Surgery, Achilles Tendon Release and Reattachment.

Sleep study test and lung function test a month later to check for respiratory issues. All was well!

Under the assumption of Sonya's Campomelic Dysplasia diagnosis, we started seeing an orthopedist at CHOP for her clubbed foot. The first attempt to correct it was to do a series of casts which would slowly stretch her achilles tendon into it's correct position. If this would not work then surgery would be needed. The day after my dad passed away we headed down to CHOP to have Sonya's first cast applied. I know my dad would have wanted me to still get to this appointment, it was important. Unfortunately after a month of this series of casts method her doctor found that it was not working and Sonya had her first surgery on May 9th 2008. An achilles tendon release surgery was done to correct her clubbed foot. She was put into a cast for a month and then she was braced with a Mitchell Bar Brace, setting her feet outwards at a 70 degree angle. If I only knew how damaging this could have been with her Osteogenesis Imperfecta diagnosis! I did decide on my own that Sonya was done with the brace after only 4 months. I just felt like something wasn't quite right and that I should stop putting it on her.

Sonya with her Mitchell Bar Brace to aid in the final correction of her club foot.

Extreme flexibility huh?!

Family vacation to Brigantine Beach NJ.

Sonya loves her mommy!

9 months old.

Sonya's 1st Christmas!

Opening presents!

Sonya turns 1!

Birthday girl and her cake!

My three girls January 2009.

For the first year of Sonya's life we assumed her diagnosis was Campomelic Dyplasia. When all 3 of the DNA tests for it came back negative over the course of the first year, her geneticist finally sent out the first DNA sample to be tested for Osteogenesis Imperfecta (his second guess). Well, within a month it came back positive for type 3! Man, was I angry that this test wasn't sent out with the tests for Campomelic Dysplasia. We could have known this when she was just a month old, special precautions and treatment could have started much earlier and eliminated much of her pain that she endured all that time! I digress, our Sonya finally had a diagnosis, but what did this mean?!

Osteogenesis Imperfecta (OI) is a genetic disorder characterized by fragile bones that break easily. It is also known as “brittle bone disease.” A person born with this disorder is affected throughout his or her life time. In addition to multiple fractures people with OI often have muscle weakness, hearing loss, fatigue, joint laxity, curved bones, scoliosis, blue sclerae (whites of eyes are blue), dentinogenesis imperfecta (brittle teeth), and short stature. Restrictive pulmonary disease occurs in more severely affected people. OI is caused by an error called a mutation on a gene that affects the body’s production of the collagen found in bones, and other tissues. It is not caused by too little calcium or poor nutrition. OI is variable with 8 different types described in medical literature. The types range in severity from a lethal form to a milder form with few visible symptoms. The specific medical problems a person will encounter will depend on the degree of severity. A person with mild OI may experience a few fractures while those with the severe forms may have hundreds in a lifetime. The number of Americans affected with OI is thought to be 25,000-50,000. The range is so wide because mild OI often goes undiagnosed. The majority of cases are caused by a dominant mutation to type 1 collagen (COL1A1 or COL1A2) genes. Other types are caused by mutations of the cartilage-associated protein (CRTAP) gene or the LEPRE1 gene. This type of mutation is inherited in a recessive manner. OI occurs with equal frequency among males and females and among all racial and ethnic groups. Approximately 35% of children with OI are born into a family with no family history of OI. Most often this is due to a new mutation to a gene and not by anything the parents did before or during pregnancy (this is the case with Sonya). Since 1979, OI has been classified by type according to a system based on mode of inheritance, clinical picture, and information from x-rays. The characteristic features of OI vary greatly from person to person, even among people with the same type of OI, and even within the same family. Not all characteristics are evident in each person. Health issues frequently seen in children and adults who have OI include: Short stature, weak tissues, fragile skin, muscle weakness, and loose joints, bleeding, easy bruising, frequent nosebleeds and in a small number of people heavy bleeding from injuries. Hearing loss may begin in childhood and affects approximately 50% of adults. Breathing problems, higher incidence of asthma plus risk for other lung problems and curvature of the spine. To read more you can check out my Osteogenesis Imperfecta post.

Sonya has type 3 moderate Osteogenesis Imperfecta. In most information that can be found on OI, this type 3 is defined as the most severe among those who survive. Sonya has been classified as moderate by her specialists. She is not one of the mild cases yet she is not among the most severe, she is somewhere in between.

Upon finding her new diagnosis I found new doctors, *SPECIALISTS* for Sonya, at AI duPont in Wilmington DE, where they have a OI clinic, and we love them! They have wonderful knowledge of Sonya's condition and we trust them to do what is best for Sonya. There is no cure for OI, but there is treatment. Sonya is on a bisphosphonate called Aredia Pamidronate (PAM). It is an IV infusion that is strengthening her bone density and lessening the amount of the fractures she may sustain. She receives this every 8 weeks for 3 days consecutively for 4 hours a day. For more info you can read my Pamidronate post.

The princess and her PAM!

We carry with us at all times Sonya's diagnosis papers to avoid allegations of child abuse and a "break box" chuck full of splinting materials, pain meds and severe trauma first aid items. Sonya has had 13 fractures to date (she is 20 months old). Both her femurs (thigh bones), left tibia (lower leg) and right radius (lower arm) broke while she was in utero and she also had 2 spinal compression fractures while in there. Shortly after birth in between transport from the local hospital to the CHOP NICU she had another 2 spinal compression fractures. All of these broken bones plus her extensive (over 100) wormian bones (extra suture lines in the skull, there are typically 6) went unnoticed by her doctors at CHOP, explaining the misdiagnosis for over a year (yet I'm still disappointed in their care and examinations, how do you miss 8 broken bones and a funky looking skull on x-rays?). She broke her right radius and ulna (lower arm) in March 2009, her left femur in June 2009, her left index finger in August 2009 and she had a micro fracture in her left scapula in August 2009. Yet through it all she remains strong and happy!

March 2009

March 2009... same break, different cast, she took off the first one!

Sonya with her new SAM splint!

June 2009

She never ceases to amaze me with her determination and strength, her courage and knowledge, her spunk and beauty! We take it all day by day. I often get asked how I do it all, how do I deal with all of this? My answer always stays the same... "A crap load of coffee, incredible patience, next to no sleep and because I have to, I love her! When you become the parent of a special needs child there are extra steps and necessities, setbacks, stress and anxiety but all in all when it comes down to it you get yourself out of bed and work with what you have because you have to, you love your child!" Sonya is a blessing! I am thankful everyday that I got to keep her! Oh, and life is still good, very good!!!

With mommy on the caurosel, no casts or breaks for now!

Sonya's Big Girl Bed!

noreply@blogger.com (Melissa Swartley) — Fri, 25 Sep 2009 01:47:00 +0000

Ok, so I thought this wouldn't be happening this soon (she's only 20 months old) but we were having problems with Sonya being in her crib. The past few weeks have been nerve racking in the mornings! She is so quiet when she wakes up that I never know when or even if she is awake! Sonya is pulling up to stand again, which is great, but she was attempting this in her crib and was loosing her balance and smashing her face into the rails. This is my best guess as to what was happening. I would always enter her bedroom to see a screaming Sonya with her fists clutching the rails, legs crunched under her body in awkward positions, her face smooshed into the rails lips askew against the wood and her neck bent back so far it looks like it might snap. I've also entered her room and seen her legs wedged between the rails and stuck! I used to use a bumper guard but I had to remove it about 6 months ago because Sonya would put her head in between it and the rails and would get stuck that way! It looked like a suffocation hazard! My older girls didn't move into their big girl beds until they were closer to 3 years old. But Sonya looked like she was going to do some serious damage to herself in the crib! The last thing we need is for her to break a bone just from being in her crib! So, it was time for the big girl bed!

Because of Sonya's Osteogenesis Imperfecta she can not sleep in your average twin bed or even toddler bed! The chances of her falling out of bed at this age are pretty high, even with guardrails... she would probably climb those and somersault her way out! A fall from any height, even just a few inches could be harmful to Sonya. So to avoid more fractures we made up a bed the OI way! It's actually a Montessori method. Well, we went to IKEA and picked up a full size Sultan Tarsta Pillow Top (no mattress) and slipped a waterproof mattress cover and fitted sheet over top and laid it down on the floor for her! The pillow top is very comfy, yet thin enough (1 & 5/8" thick) so that if she were to roll off of it onto her carpet she wouldn't even realize it! Of course I had to OI child proof the room then, but it wasn't too bad. The only major thing I had to deal with was the bookshelf, I just moved it into her closet and closed the doors. The rest of the room was simple... remove floor lamp, plug up the outlets with safety shields, move dresser to cover up an outlet that was accessed for CD player and monitor and gave the carpet a good vacuuming and check over for small choking hazards. We still want to get an anchor for her dresser just to be extra safe, but we think it is fine for now. She has a very old vintage dresser from the Victorian era and it weighs a ton! I can barely move it myself! To dress it up a bit, I added super cute animal vinyl wall rub-on's from Michael's Craft Store to the wall that her bed lies against. I figured it made up for the non-existent headboard, foot board and ummmm bed! Her first night sleeping in her big girl bed was Tuesday the 22nd! She did great! She was a little scared and worried at first, but 10 minutes lying down with mommy in her new bed with some calm talk about this different look and feel was all it took! I left the room while she was still awake and I presented to her a new little stuffed webkinz kitty cat for extra comfort which she loved! All went well and I am hoping and praying that the rest of the week continues to go just as smooth!

Here she is today after a long night last night in the ER. Can you see the bags under her eyes?!

She is loving baby dolls right now!

Here's the new bed complete with my king sized (it's folded) quilt that my grandmother and I made (mostly my grandmother).

Sonya's kitty cat and cute animal wall vinyls are waiting for her!

Zebra, hippopotamus, giraffe and elephant (Sonya's favorite).

Tiger, lion, hippopotamus and zebra.

The new room!

Sonya loves the animals!

"Hi, ippo"

Rolling around, testing it out!

"Ooops!" She's on the carpet! It was a smooth transition though!

Akela and Zoe are so proud of their little sister and her new bed!

"Can we sleep in here too? It's soooo cool!"

New milestone this week: Climbing! Everything and everywhere! She's unstoppable!

She climbed the whole flight all by herself!

Nothing is safe... not even the Bounty paper towels!

This cute rocking horse seat used to be mine when I was a toddler. My dad refinished it when Zoe was born and all three of my girls have enjoyed it! Sonya loves it, but she doesn't like it when I tell her not to stand in it... "Not safe, Sonya!"

Being a monkey, I don't like the positioning of her feet when she does this! Her left foot is twisted around backwards! Just noticed that!

"Waaaa!!!!" Mommy told me to sit down and behave! The terrible two's are on our doorstep!

Sonya's Almost Skull Fracture

noreply@blogger.com (Melissa Swartley) — Thu, 24 Sep 2009 18:04:00 +0000

Sonya had a bad fall yesterday evening around 6pm while she was trying to climb our end table in the family room (this is her favorite spot and I am starting to feel like I want to get rid of it :0 ). Anyways, she pulled out the bottom drawer, pulled up to a stand while in it and then lost her grip, fell and knocked her head real hard on the table and then the drawer as well. She cried hard for about 10 minutes and then calmed down and started playing again, being her usual self. I didn't think anything of it since she seemed fine again, but then later last night she gave me the worst time going to bed, started holding her head and screaming "Boo Booooo!!! Ow Ow Ow!!!" She is always the perfect angel when it comes to bed time so this really worried me. I felt her noggin and found a giant egg shaped lump right where she had hit it earlier. Man, did I feel stupid for not taking her to the ER right away!

I called my husband and had him drive 45 minutes home from work at about 1am (he's a 3rd shift worker) to be here for the older girls who were tucked in bed asleep and then took Sonya in to the ER by myself. The place was empty... thank God!... but apparently there was an insane rush of people just before we arrived because it took them a half hour to clean up a room for us! The on call doctor, who has seen us for every situation so far came in quickly and gave Sonya a sedative (Ketamine and something else) to calm her down for the CT scan. Sonya was soooo loopy and fought sleep the whole way! She even kept raising her hands and arms up as if to say "no" while she received her CT scan! Talk about strong willed!

The scan results came back after about an hour. They send all of their tests out electronically to a place in Missouri that has a few hundred Radiologists sitting there reading them all and they then send the results back. There was no fracture! She must be hard headed as well as strong willed! When all was said and done we finally went to bed together at about 5am. I'm exhausted as I had to get up to get Zoe off to school this morning at 7:30am. Sonya got up at 8 this morning all cheerful and is now zonked out on the family room floor taking a well needed nap!We are supposed to follow up with our ortho, I will be calling them shortly.

Zoe's Eco-Friendly Birthday Bash!

noreply@blogger.com (Melissa Swartley) — Mon, 21 Sep 2009 04:03:00 +0000

For Zoe's 8th birthday this year she wanted to do an environmentally friendly party! Lately she has become very Earth conscious and has been asking me about everything "green". I'm so excited and proud that my big girl has great values and ideals when it comes to nature and sustaining our planet!

One very "green" chic! Oh yeah, Zoe got her hair cut for her birthday too! She almost had enough to donate! She was shy by one inch! Oh well, maybe next time!

Ok, this is literally the best shot that I got of all 3 of my beauties this Saturday... Hmmm.... looks like we need to practice our photo shoots a bit more! Sonya is watching Elmo on TV, Akela is looking God knows where and Zoe is completely irritated that I have already begun with the picture taking, not to mention she has a pole coming out of her head!

Akela being cute, but my camera is totally on the wrong setting and the lighting is bad!

"Sonya bean" being too cute!

I love this picture of her!

Zoe's Earth cake that mommy made. It's actually not cake, but 2 layers of homemade brownies topped with french vanilla frosting (dyed with food coloring).

I think Zoe got a slice of Africa.

The cake was for our family party 11am-2pm and then I made homemade brownie cupcakes for the kids party 2:30pm-5pm. I used silicone bake cups to keep with the "green theme".

So, Ian and I totally splurged and got her a Nintendo DS Lite with 2 games and carrying case. She was soooooo happy about this! The only thing "green" about this is it's color and the amount of green I had to drop to get it! Here is Zoe playing (totally addicted to video games already) and her sister Akela and cousin Camille checking it out on the sidelines.

Zoe also loves everything having to do with cats! She is completely obsessed! Other than all the cat stuff that she received she also got a beautiful locket with her initial "Z" on the front, some really cool magic tricks and a pretty bird feeder, American Girl Doll outfits, a savings bond and build a bear clothes, a sequins purse, Pokemon cards, lip gloss, nail polish, eye glitter, a dry erase board and picture frame, $ and a cute ballerina bear! Here is a picture of Sonya enjoying some of those cute cats! Shh! Don't tell Zoe, but I'm totally letting Sonya play with these while she is in school!

Here is Zoe with the book "Just Grace Goes Green"

Little miss Sonya hanging out with my sister Meghan, Aunt Mae Mae.

For the kid party part of the day, we kicked it off by making jar lid photo magnets (paint a jar lid, cut photo to fit and glue it inside then hot glue a magnet on the back). We then made bookmarks out of cereal boxes, each kid picked out their favorite box (I've been saving for months). The next project was to plant crocus bulbs inside origami paper pots (biodegradable) and I sent them all outside with Ian to do so, so that I could sew up their t-shirts that they brought with for crafting into tote bags! Inside their tote bags I also placed the fun snack bags that I made out of potato chip bags! Here is Zoe all proud of her beautiful planting project!

What September "green theme" party wouldn't be complete without some bobbing for granny smith apples?! The only downside is that because it is apple season here in Pennsylvania, those suckers are huge, pretty much too huge for little 8 year old mouths to grasp! All of the kids were happy to try and then take one of the apples to snack on while swinging on our swing set out back.

Zoe giving it her best shot!

After the bobbing I had all the kids pick out some "green theme" iron on patches and buttons for their t-shirt totes and I put them on. I then had them help fill a ice stick tray (the kind for water bottles) with broken junked up old crayons that everyone had brought and that had been peeled of their papers. We sorted the colors and then melted them down in my oven into new whole crayons! Each kiddo got to take home 2 new pretty crayons! Then came the really fun part! I saved up wide mouthed vitamin water bottles, cleaned and dried them out really good prior to the party. I gave each of the kids a bottle and had them promise me and Zoe that they would recycle the bottle after they finished what was inside. They promised us but looked very puzzled. "But Mrs. Swartley, there isn't anything in the bottles?!" That's when I picked up the huge shopping bag full of a variety of candy and dumped the whole thing on the kitchen table and told them they had 2 minutes to pack their bottles as full as they could get them! They went NUTS! I wish I captured more pictures of all the excitement, but I really had my hands full with this wild bunch! P.S. That is Zoe's unofficial boyfriend sitting next to her. She won't admit it, but they are just too cute together! Happy 8th Birthday Zoe! I love you soooo much!

Side Note : This is what Sonya thinks about her activity table....

What a goof!

Stay tuned for Sonya's Big Girl Bed post coming soon!

Watch This!

noreply@blogger.com (Melissa Swartley) — Wed, 09 Sep 2009 13:46:00 +0000

Growing Up Tiny : Kenadie's Story on TLC Sunday the 13th at 8pm Eastern Standard Time. I saw Dr. Bober (Sonya's doctor) in the commercial preview for this! I didn't know that he see's Kenadie! Anyways, watch it! Kenadie is such a sweet and inspiring little girl!