Update On Sonya's Teeth

Last week I put a call in to see Dr. Inga at Dupont whom we were referred to and who specializes in treating kids with Dentinogenesis Imperfecta (DI,brittle teeth) secondary to Osteogenesis Imperfecta. DI is not decay or anything like it, it's a genetic condition and cannot be resolved by any amount of extra cleaning or the like. The only treatment for this problem is sealing, bonding, capping and crowning. Essentially the same collagen defect that causes her dwarfism and her bones to break also causes her teeth to wear done, chip and break away in a painful manner. All this happens just as fast as her teeth start to come in. The dentist office had called me back on Friday just before they closed at 5pm to set up an appointment, however I unfortunately missed their call. So I planned on calling them back this Monday, but I totally forgot and only remembered yesterday to do so. I happened to be lucky yesterday and called right after they had a cancellation for today so they fit me in at 11 am this morning!

Well, we went to see Dr. Inga at Dupont and we were told that Sonya's teeth are not fully erupted yet and that her clinical crown (found by gliding the dental tool along and up the side of each tooth, it's where the root begins) isn't evident yet so we will have to wait until they are in all the way before we proceed with sealing, bonding, capping and crowning. Without the clinical crown the sealing, bonding, capping, or crowning will not stay put and would just fall off with her first bite into something yummy. I'm really bummed about this since her two upper front teeth have worn down significantly already to the point where they are almost gone. I was really hoping to walk in to the dentist's office and be told to set up an appointment in the next few weeks or so for the procedure. Her upper two front teeth broke through her gums at 8 months and were a good size. Here we are 7 and 1/2 months later and they are more than half gone from wear and they are not through all the way?! There's no way to determine how long it will take for them to fully erupt either! I'm frustrated by this and super worried that her teeth will wear down completely before they fully erupt! Then what, she'll have to go back to eating baby food until her permanent teeth come in at age 7?! Grrrr!!! She did have her teeth cleaned and treated with fluoride while we were there, which she absolutely hated every minute of. We will be going back in 6 months for another cleaning and fluoride treatment and to see how things look as far as the clinical crowns go. Thanks for listening to me vent!

Here are some photos of Miss Cutie Pie driving her new car! She loves going for long walks around the block and honking her horn at everyone she passes!

Sonya loves her new ride! It's just the perfect size for her and it has a seat belt for safety too!

"Hey! No tailgating me!"

Here she is checking out the birds flying overhead.

Just being cute!

All tuckered out in her highchair after a long day of play and dentistry!

How does she sleep in this position?!

Sleeping Beauty.

Beautiful Weather

Well, we finally have some nice weather here in northern Pennsylvania! It was cold and rainy in the mid 40's to low 50's up until this weekend when all of a sudden we spiked at 92 degrees! I can't stand how wacky PA weather can be! In the past few years we have been going from one extreme to the other and skipping Spring altogether!!!! My tulips had just sprung up and were ready to bloom before the heat arrived and now they are all bloomed and wilting! Grrrrr.... Oh well, at least the kids are enjoying the fresh air, humid as it may be, and the inside of my house is a little bit more in order since they haven't been inside shredding it up! LOL! Here's a few pictures of Zoe, Akela and Sonya chillin' by the flowers. Zoe has a major fear of bees now after she was stung over at her Nanny's house, and of course with all of my flowers in the garden now the bees are a plenty! I tried calming her down a bit and telling her about my allergies to bee stings, but that I didn't let that matter keep me locked up inside all Spring, Summer and Fall! She's still running away from the bees! Poor Zoe!

Sonya says "Ok that's enough pictures already!"

After checking out Simon's blog and seeing that Simon was making a phone call to Sonya, she was so excited she grabbed up my new phone (the Samsung Impression, my 4th Motorola Razor kicked the bucket yet again) so that she could talk to him. She was telling him all her new words! This was just so cute I took some photos! I wish I had video of it! Sonya now says : hi, bye, pretty, cheese, juice, mama, dada, Zoe (So-he), Akela (Ka-ya), meow (when she see's a cat), yum, uh-oh, that (whenever she wants to know what something is, she points and says this), haaaa (very exaggerated, whenever she knows she's being clever and you have caught her in the act), and Bob (Ian's Uncle Bob, she says this all the time even while not in his presence... she likes your name Uncle Bob!), and Si (for Simon)!

Talking to Simon... "Uh-oh! Bob.... Meow! Pretty... Mama! Si!

"Cheese!, Yum, Uh-oh!"

"Si..... Si..... Siiiiiiii!!!!!!"

She is so cute with all her babbling now! I will be finishing up all the gardening work this week so hopefully I will be able to post some pictures of all our hard work soon! Everyone enjoy your week!

So, there's a lot more to Sonya than we thought......

Our appointment went very well today with Dr. Bober at Dupont in Wilmington DE. We got in to see him at 1pm and didn't leave until around 4pm! He was very thorough and went over everything dealing with OI as if I was hearing it all for the first time. Sonya took to him nicely too and let him check her out with no complaints!

Well, here's the not so good news... There is more to Sonya than we previously thought. Apparently our previous doctors at CHOP failed to tell us some things or didn't look closely enough at her x-rays. It turns out that Sonya has had fractures in the past and worse, she's had spinal compression fractures. Both of her femurs (thigh bones), her left tibia (lower leg bone), and her left radius (lower arm bone, not the one that's casted now) have all been broken in the past, most likely while in utero. While examining her x-rays Dr. Bober noticed many compression fractures in her spine (this is not good). He discussed with me how to recognize a compression fracture in the spine on an x-ray. Typically the spinal column looks like a bunch of nice large stacked marshmallows, but with OI spinal compression fractures the bones look like flattened marshmallows. I saw a bunch of flattened marshmallows.... I counted about 10. Dr. Bober wants to see her x-rays from birth (CHOP failed to put them on the CD that I requested and only her new x-rays from last week were on there) to see if the spinal compression fractures were there at birth. He put some pressure with his fingers up and down her spine and she didn't react, which is good! Finding out when these compression fractures occurred is important because we will have a better idea of how severe Sonya is. Dr. Bober says they are most likely are old fractures and I am sooooo hoping thy are! All the old breaks including the spinal compression fractures is new news to us! I'm slightly shocked, but then again OI is what it is and this is to be expected... lot's of fractures.

Dr. Bober has told us it is not the type (3/4) of OI that matters, but the severity. This is something that I had known, but I didn't know where Sonya was on the severity part so I always referred to her as type 3/4. Sonya is not on the mild side or severe, she is moderate (just in between). The severity is determined by the way the fractures heal. Sonya's bones do not heal straight but rather angled a good bit.

Sonya has major markers for OI that her doctors at CHOP did not pick up on at birth including her blue sclera (whites of eyes are blue), extensive wormian bones (lots of little extra bones in her skull, nothing to worry about, just a big indicator of OI and something that I had never heard of before today) and the way her pelvis (hips) are situated are very characteristic of OI. Dr. Bober says he is surprised that they didn't pick up on her having OI and not Campomelic Dysplasia (CD, her previous diagnosis at CHOP) as it is very obvious! Imagine that!

So after our evaluation we discussed treatment plans. Pamidronate (PAM), as I have blogged about before is a bisphosphonate used to treat OI. It is not a cure and at it's best it will strengthen her bone density and prevent fractures but not all of them. She will still break, just not as much. Right now we are going to start to build up her foundation of calcium and vitamin D. The two work hand in hand, vitamin D helps the bones absorb the calcium. Without sufficient vitamin D the calcium Sonya ingests just gets pooped out. So we went to have her vitamin D blood test to see where her levels are. If she is lacking the proper amount of D, which 80% of the American population is lacking by the way, she will be put on supplements (this is most likely). We need to ensure that Sonya is also getting the proper amount of calcium by means of diet. Whole milk, yogurt and cheese were big suggestions as we strive for 500 mg a day for her. She loves, loves, loves yogurt and cheese and is starting to drink milk pretty well too, so this should be easy! After we regulate her diet and get used to this new intake she will be ready for PAM. Although, Dr. Bober believes she is in the gray area for now as to whether she will need it just yet. It is in the future however. We also discussed the suggestion of having her get a port to ease injections. The drug would be administered every 6 weeks, over the course of 3 days (4 hour infusions a day) at the beginning and then we would eventually be able to spread her doses out to every 8 weeks then possibly 10. PAM is a long term commitment and once Sonya starts she will be on it until she is finished growing (around 15 years old). The benefits of this drug greatly outweigh the risks involved. Most of the risks are things that occurred in studies with cancer patients. Most likely Sonya will only experience flu like symptoms for a short duration following treatment.

As far as the rodding surgery goes, the protocol is 3 long bone breaks (anything but hands and feet) in 2 years time and it's time to rod! Sonya has one down. Even though she fractured 2 bones in her arm this time, they consider it one break because it was one incident. Femur and tibia rodding is very possible for Sonya in the near future as she has been weight bearing and pulling to stand. We were forewarned that as she learns to let go and take those first steps the possibility of fracturing her legs is very likely.

We also discussed Sonya's Dentinogenesis Imperfecta (DI, brittle teeth) and guess what? The good news is that Dupont has a pediatric dentist that treats all their OI kids with DI there! So we will be setting up an appointment there ASAP! Yay!

Going over our Physical Therapy (PT) and Occupational Therapy (OT) information Dr. Bober felt it would be a good idea to see their therapists at Dupont to have an evaluation and then have them work with our current PT and OT. Really good news ~ the therapists at Dupont have lot's of experience with OI kids! Dr. Bober would also like to see Sonya getting some swim therapy such as a mommy and me swim class to start.... once the cast comes off of course!

Geez! That was a whole heck of a lot of info to take in! We are all doing good with the new info and thank you all for your support and prayers! For added fun I uploaded "the scream!" This is Sonya's loud enough to give you the worst headache ever scream! Mind you she does this for fun most days! In this video she is mad that she ate all of her freeze dried strawberries and we are all out! Too funny!

Sonya's Medical ID Bracelet

Because of Sonya's tendency to break easily we decided to get her a medical ID bracelet just in case something horrible should happen, God forbid. If we were in a car accident or any other similar situation where Ian and I would be unable to speak for her (knocked out unconscious ect.) whomever arrived on the scene would need to know that Sonya has OI and to handle her with extreme care as well as get her checked out a bit more than the standard. We ordered her pretty pink heart beaded medical ID bracelet for her off the Internet at this site http://www.petitebaublesboutique.com/ . We just got it in the mail the other day! It's so cute!

Front Says:

Sonya

Swartley

DOB : 1-16-08

Brittle Bones

Back says :

Osteogenesis

Imperfecta type 3/4

Please handle with

extreme care!

Now I can rest a little more easy. I still want to add some kind of tag or sticker to her car seat as well for a bit more information. If anyone has some ideas let me know! I have to put it where it will be seen, yet somewhere that Sonya can't rip it off and eat it! LOL!

So this video is Sonya freaking out because she doesn't want to say goodbye to her buddy Simon! We watched Simon's new walking video clips for about 15 minutes over and over and over again! She just loves her Simon and she is soooo impressed with his walking skills and unbelievable speed! So when it was time for mommy to get off the computer and start getting some housework done, Sonya had a complete meltdown and went through Simon withdrawal!

Too cute! Don't worry Sonya, I'm sure you will get your Simon time again soon! By the way Jenn, this is almost half as loud as her "I wanna hear myself big screech"! I'm not sure if a video clip would even do that scream any justice! She can get super loud, I wonder what the neighbors think? LOL!

hOpPiNg DoWn ThE bUnNy TrAiL!

Happy Easter Everyone!!!

Haha! So much for getting all three of them looking at the camera! Oh well! At least there were no tears involved! Don't you just love Sonya's "Uh Oh!" face? Yep, that's her new favorite thing to say now, replacing "dada". Everything is "Uh Oh!"

My fiestaware cups are perfect for knowing which dye is which!

Zoe holding up her egg before she dunks it into her favorite color, pink!

Somebody didn't get a long enough nap and could care less about dying her egg her favorite color, blue!

Now Akela is really angry because I took her sad face picture! Needless to say, egg dying was a short and sweet procedure.

The bunny came!!! He even left his white fluffy tracks!

Sonya doesn't know what to make of the tracks!

To create the bunny tracks I used baby powder and sprinkled it on our carpet in a hopping footprint pattern from our front door to the kitchen where the girls Easter baskets await. They love coming downstairs in the morning to see if the Easter bunny left his tracks! They always say, "boy that Easter bunny sure is messy mommy!" and then they stomp out his tracks, powder dissipating in the air. Just a little vacuuming finishes the job.

The girls were very happy to see their full baskets! All pink candy for Zoe, blue for Akela and a mixture of fun things for Sonya.

Sonya got her first Little People toy! She has been playing with her sister Akela's Little People toys for awhile now and loves them! Now she has her own car, mommy and baby!

Zoe loves her big bouncy ball with pink glitter inside it!

Akela was so happy to get blue Peeps this year!

My three happy girls on Easter morning!

5 Appointments Accomplished!

Yep, that's right! We had 5 appointments already this week and we have more to come next week! It has been getting quite busy lately now that Sonya has her diagnosis.

This Monday we had our first Occupational Therapy appointment and it went very well! The therapist was very happy with Sonya's playing skills. She would like to keep seeing her once a week for at least a few months, but she feels that Sonya is doing very good and will benefit more from Physical Therapy.

Tuesday we were initially scheduled for Sonya's first Physical Therapy appointment, but plans changed when Sonya had broken her arm. We needed to see her OI specialist on this ASAP! Of course Sonya decided she would attempt to make this more complicated and she ripped off her cast Monday night! That's right, she ripped it right off as if it were nothing! What a monkey! Of course she did this after all of our doctor's offices were closed for the day. We did manage to get some messages back from our support group telling us to wrap her arm up in ace bandages and then wrap another ace bandage with her arm and hand pledge of allegiance style, securing the wrap around her belly. "Sonya Houdini" managed to keep Mommy's do it yourself home bandaging on through the rest of the night and through the morning until we met her OI specialist Dr. Kruse, whom we are very happy with. After Dr. Kruse had her x-rayed, he determined that she was still healing and he had her re-casted for 2 more weeks. We got to talk a little bit about some of our immediate concerns, her Dentinogenesis Imperfecta, possible rodding surgery and Pamidronate treatment, but since we were squeezed in this time under special circumstances (broken arm) he said we would talk about these issues more in depth in 2 more weeks when we come back to be uncasted. He did suggest that in the meantime we set up an appointment with Dr. Bober, a geneticist/ OI specialist who will be able to discuss Pamidronate with us as well as a few other concerns we have. The two of them work very close with each other and some of the OI specialists at the Kennedy Krieger Institute down in Baltimore. We have the appointment all set up to see Dr. Bober on Thursday the 16th and we are very excited to meet him!

Today (Wednesday) we had appointments 3 (audiology), 4 (full body x-rays, skeletal survey) and 5 (Ortho/ Pamidronate/Bone Density Dexa Scan). Sonya's hearing screening was great! She does not appear to be having any issues with hearing and there is no fluid issues either! YAY!!! We were referred to speech therapy though, as she is a bit behind in verbalizing. We will have to tack this on to the list of upcoming appointments! After Sonya's hearing screening we went over to radiology and had her skeletal survey (full body x-rays) done. Sonya completely flipped out, turned beet red and pierced everyone's ear drums with her siren scream. She fought us the whole time and it took 3 people, myself included to restrain her for her pretty bone pictures! The good news is that I requested a CD of her x-rays, so now when we see anyone outside of Children's Hospital of Philadelphia and they want to see her x-rays I will have a disc for them to copy instead of having to have Sonya repeatedly undergo this exhausting torture! Hopefully this will be the last set of x-rays for the next 6 months excluding any fractures she may sustain.

After the torture chamber... errrr... x-ray room we headed on over to the "bone doctor". This is how he has been referred to for me and I thought he was yet another orthopaedic however on his business card it says Endocrinologist. The appointment with this doctor went less than smooth to put it lightly. After he introduced himself the appointment went downhill faster than a jack rabbit being chased by a cheetah. I felt muted as he asked me questions expecting only one worded answers to spew from my mouth. I initially tried to introduce Sonya with a simple brief of her history but I was cut off sharply with a "wait, wait, wait, wait just a minute..." as if my short excerpt was too much for his ears to hear and brain to absorb and he rushed over to his computer to quickly type down more than what I had begun to say.

"What makes them think she has OI?" he asks.

"Uh, she was clinically diagnosed and we have the DNA tests to prove it." I answered, dumbfounded. Did this guy even read any of her history while he was typing his novel during my one worded answers? Let alone look at her, the almost 15 month old with bluer than blue sclera (the calling card for OI) and her now below one percentile height and weight growth. Run now.....

"So, I assume you are familiar with the drug Pamidronate?" he asked.

"I've heard a bit about it but not everything" I answered.

"Ok, well then I propose she be put on it immediately." he quipped back.

About a 5 minute pause lingers here.....

"Well, would you be doing a bone density Dexa scan first to see where she stands?" I inquired, thinking wow he's ready to jump the gun!

"Well..... yes.... eventually and we will have to do many more blood tests to determine if she should be on the drug." he answered hesitantly.

Another 2 minute pause.... He isn't going to start talking about this, I'm thinking?

"Ok, what tests are we talking about?" I asked.

"Vitamin D blood tests, urine creatinine, urine calcium, phosphorus, thyroid tests to make sure her growth restriction isn't something else....among others" he quickly replied with no explanation of the tests.

Another awkward lengthy pause.... Seriously are we going to converse here?

"What are the risks involved with Pamidronate? I know that it has only been in use for kids with OI for 10 years so the long term effects are unknown, but what about the other risks?" I offer up, seeing as how this guy isn't going to be offering any information without my inquiry.

"Kidney failure..... But mostly that happens with adults who have been using this drug with cancer treatment..... The kids usually just have flu-like symptoms right after their infusions." he answered.

"Woah, kidney failure?! Wait, wait, wait, wait just a minute.. (now it's my turn) were you going to explain any of this before you put her on the infusions or were you going to just see how far you could get with her before I would ask these questions?" I put him on the spot.

"Well we will be having more meetings to discuss all of this before she starts her treatment." he looks up at me finally and acknowledges my presence.

His pager starts to go off and he quickly excuses himself leaving the room and us in his dust. I sat there with Sonya thinking, ok just get through this appointment without completely going ballistic on this crappy doc. We have an appointment with a very favorable Dr. Bober for next Thursday and we can just blog vent this wacko and never return. Thirty minutes pass..... Thirty!!!!! This guy is a joke! He finally returns just as I have finished packing our bag up and was literally ready to reach for the door knob. Do we get an apology? Of course not, this guy is a jerk! He walks straight over to the computer with the silence of a mouse and starts pounding keys again. I think my ears had steam shooting out in billows! He handed me the paperwork for all of her lab work to be done and directed me to the blood work testing lab. Instead we just left for home. How disappointing!!!! I was so glad that 76 was smooth sailing (30 minutes sitting in standstill traffic and then proceeding at only 45 mph is smooth for 76) because if I had to deal with anymore idiots for the day I probably would have had a nervous breakdown! I love you Philly!

So, 5 appointments down and many more to go! Tomorrow we will be seeing Zoe's dentist as her top front permanent tooth is coming in over her primary tooth that won't wiggle itself out! Zoe is soooo worked up over this and completely scared to death of the possible pulling of her baby tooth. Monday we have Sonya's second OT appointment, Tuesday we have her first PT appointment and Thursday we will see Dr. Bober. The following week there are 6 appointments. I really haven't peeked at the following week, but I know deep down it's busy too. Well, that's it for now... Here are some pictures of my little Houdini!

One handed fishing with Mommy's do it yourself bandage job.

Not too shabby eh?

Entering the city of brotherly love.

Otherwise known as Killadelphia or Filthadelphia, we love you anyways Philly!

Sonya and her pretty new pink cast!

Playing with her Little People.

Yep, she's still getting around just fine!

My little cutie!

I made this video right before Sonya decided to remove her nicely signed cast. This is her "hermit crab crawl" as we call it. Please excuse Akela's crack, I need to get this kid some belts!

Update On The Cast.....

Ok, so many of you already read my short blurb on Facebook about what happened with Sonya's follow up appointment for her cast with the local ortho. Now let me indulge... errrr... horrify you a little bit more with the details....

I knew we had to find a OI specialist for Sonya only a few weeks before her first and second breaks (ulna and radius at the same time). I had no clue where to start and when faced with the choices they were tough. I figured we had time to think about all of it and weigh out the pros and cons. We had two really good options. The Kennedy Krieger Institute (KKI) in Maryland that sees 250 OI patients a year (the most in the USA) and is 3 and 1/2 hours away from our home or A.I. duPont in Delaware that sees 75 OI Patients a year and is 1 and 1/2 hours away from our home. While KKI has more OI experience it would be nice to only have to drive an hour and a half since we will most likely be going very often (I'm talking weekly to bi-weekly or monthly depending on how she does with breaks). That being said we chose Dr. Kruse at duPont. Unfortunately since we are new patients we can't get in until June 1st. Even more unfortunately I was wrong about having time to decide where we would go for treatment. Sonya broke and our only option was to go to the ER and follow up with Dr. Lin, the ortho who handled her case at the hospital, short of calling Dr. Ain who had already referred us to KKI for her OI. As you may all recall I was pretty happy to have good ortho Dr. Lin just down the street.... I'm still happy with his medical expertise but I am not at all enthused with his lack of bedside manner.

Well, we went to go see Dr. Lin for a casting follow up appointment on Tuesday and Sonya had her broken arm x-rayed. She was not too fond of that... as always is the case with x-rays. Dr. Lin took a look at them and said that the ulna and radius were healing but not straight, more crooked like (which is typical of OI'ers, thus the progressively deforming condition). He told us the cast would most likely be able to come off in about 4 weeks. This is about where he went from an ok doctor to OMG did he just say that?! Here are his words and I quote... "It's such a shame because they (referring to people with OI) have normal intelligence and they have to be so profoundly short, so sad..." WHAT DID HE JUST SAY?! *ringing in my head* I replied... "So having OI would be more acceptable for a person with a mental disability? I think not. Also, just to inform you, most folks with dwarfism are of average or above average intelligence. You say this as if being a dwarf is a bad thing. It's a hard diagnosis to hear, but we are doing just fine with it and her stature is the least of my worries for her. I can understand you having sympathy for her aching broken bones, compassion, understanding and support for my daughter as those are all acceptable but it sounds like you are pitying her. Pity is not at all what I want for her nor what she will want when she is of age to understand it. She is destined for great things, and she will be very capable of doing whatever she wants whether she is in a wheelchair, on crutches or upright and walking with surgical steel rods inside holding her crumbling bones together." He responded with a stumbling and sputtering of "uh... uh.... I'm so sorry to offend you.." And again I replied "Apology accepted, I just want to make it clear that we accept no pity here."

I don't think he was able to come up with anything to justify what he said and maybe this was said out of complete ignorance. He wrote up her paperwork and left the room fairly quickly afterwards. I almost felt sorry for being so quick to call him out on his disrespect, but I'm glad I did. I hope I didn't sound like a bitch, as this upset me a good deal and I get irritated easily over these things. I meant every word though. Ask anybody who has a disability... They will tell you they want your friendship, your support and your understanding but never your pity. I definitely don't want Sonya all grown up and throwing a pity party for herself on a daily basis ("Oh poor me..") and hulled up into a dark hole of despair because some doc thought it was ok to pity her! She is going to learn how to make the best of life even though it's going to be quite more painful than what the rest of us have to deal with along the way.

So, I ended up with a lot of status comments on Facebook and subsequently some online chats and support group messaging over this including some really helpful ones from Sonya's OI buddies! Seems that Sonya should not be in her cast any longer than 3 weeks. With OI kids, having muscle tone protects their fragile bones! The longer Sonya spends in that cast the more her muscle tone is depleting and the more likely she will be to fracture the same arm shortly after uncasting it! Most doctors inexperienced with OI will say well, the bone isn't healed all the way and the child will have a higher fracture risk simply because of that. To this OI specialists say bologna! Damn! I sure am glad I had a "little" tiff with the local doctor or I might have never found out this valuable information! Yep, the "little" pun was intended.

Woooo!!!! There now I feel better! Thanks for listening to me vent all! You guys are the best and I love you all! Now here is some cuteness for you!

There must have been a whole lot of cuteness in this video, almost too much for Blogger to handle! Seriously, it took me 3 hours to upload it! What is up with that?! Anyways, she really loves her fishing puzzle and is becoming a pro at it!

Update On the Cast

Ok, so many of you already read my short blurb on Facebook about what happened with Sonya's follow up appointment for her cast with the local ortho. Now let me indulge... errrr... horrify you a little bit more with the details....I knew we had to find a OI specialist for Sonya only a few weeks before her first and second breaks (ulna and radius at the same time). I had no clue where to start and when faced with the choices they were tough. I figured we had time to think about all of it and weigh out the pros and cons. We had two really good options. The Kennedy Krieger Institute (KKI) in Maryland that sees 250 OI patients a year (the most in the USA) and is 3 and 1/2 hours away from our home or A.I. duPont in Delaware that sees 75 OI Patients a year and is 1 and 1/2 hours away from our home. While KKI has more OI experience it would be nice to only have to drive an hour and a half since we will most likely be going very often (I'm talking weekly to bi-weekly or monthly depending on how she does with breaks). That being said we chose Dr. Kruse at duPont. Unfortunately since we are new patients we can't get in until June 1st. Even more unfortunately I was wrong about having time to decide where we would go for treatment. Sonya broke and our only option was to go to the ER and follow up with Dr. Lin, the ortho who handled her case at the hospital, short of calling Dr. Ain who had already referred us to KKI for her OI. As you may all recall I was pretty happy to have good ortho Dr. Lin just down the street.... I'm still happy with his medical expertise but I am not at all enthused with his lack of bedside manner.

Well, we went to go see Dr. Lin for a casting follow up appointment on Tuesday and Sonya had her broken arm x-rayed. She was not too fond of that... as always is the case with x-rays. Dr. Lin took a look at them and said that the ulna and radius were healing but not straight, more crooked like (which is typical of OI'ers, thus the progressively deforming condition). He told us the cast would most likely be able to come off in about 4 weeks. This is about where he went from an ok doctor to OMG did he just say that?! Here are his words and I quote... "It's such a shame because they (referring to people with OI) have normal intelligence and they have to be so profoundly short, so sad..." WHAT DID HE JUST SAY?! *ringing in my head* I replied... "So having OI would be more acceptable for a person with a mental disability? I think not. Also, just to inform you, most folks with dwarfism are of average or above average intelligence. You say this as if being a dwarf is a bad thing. It's a hard diagnosis to hear, but we are doing just fine with it and her stature is the least of my worries for her. I can understand you having sympathy for her aching broken bones, compassion, understanding and support for my daughter as those are all acceptable but it sounds like you are pitying her. Pity is not at all what I want for her nor what she will want when she is of age to understand it. She is destined for great things, and she will be very capable of doing whatever she wants whether she is in a wheelchair, on crutches or upright and walking with surgical steel rods inside holding her crumbling bones together." He responded with a stumbling and sputtering of "uh... uh.... I'm so sorry to offend you.." And again I replied "Apology accepted, I just want to make it clear that we accept no pity here."

I don't think he was able to come up with anything to justify what he said and maybe this was said out of complete ignorance. He wrote up her paperwork and left the room fairly quickly afterwards. I almost felt sorry for being so quick to call him out on his disrespect, but I'm glad I did. I hope I didn't sound like a bitch, as this upset me a good deal and I get irritated easily over these things. I meant every word though. Ask anybody who has a disability... They will tell you they want your friendship, your support and your understanding but never your pity. I definitely don't want Sonya all grown up and throwing a pity party for herself on a daily basis ("Oh poor me..") and hulled up into a dark hole of despair because some doc thought it was ok to pity her! She is going to learn how to make the best of life even though it's going to be quite more painful than what the rest of us have to deal with along the way.

So, I ended up with a lot of status comments on Facebook and subsequently some online chats and support group messaging over this including some really helpful ones from Sonya's OI buddies! Seems that Sonya should not be in her cast any longer than 3 weeks. With OI kids, having muscle tone protects their fragile bones! The longer Sonya spends in that cast the more her muscle tone is depleting and the more likely she will be to fracture the same arm shortly after uncasting it! Most doctors inexperienced with OI will say well, the bone isn't healed all the way and the child will have a higher fracture risk simply because of that. To this OI specialists say bologna! Damn! I sure am glad I had a "little" tiff with the local doctor or I might have never found out this valuable information! Yep, the "little" pun was intended.

Woooo!!!! There now I feel better! Thanks for listening to me vent all! You guys are the best and I love you all! Now here is some cuteness for you!There must have been a whole lot of cuteness in this video, almost too much for Blogger to handle! Seriously, it took me 3 hours to upload it! What is up with that?! Anyways, she really loves her fishing puzzle and is becoming a pro at it!