Surgery is Over and Went Great

Whew! It's been a long day!  Surgery started right around 8 this morning and we finally got a call at 1:40 saying that surgery was done, she did really well, they were splinting her up, and then the doctor would be out to talk to me.  Dr. Vincent came shortly to talk to me about how surgery went.  He brought me her old rod from India, which was really interesting to see/have.  It's a generic steel rod that was supposed to be telescopic like the FD rod.  Basically, the tip on the part that goes up into the other rod portion was bent so it wasn't deploying correctly.  He had to make a cut in the bone to get the rod out, but was able to fully remove it. 

The new rods are in both her femur and her tibia and are positioned perfectly.  She has a removable spica cast on, which we won't remove for quite some time.  It's nice, though, because when she is ready to bathe I can unwrap it, bathe her, and then re-wrap it. 

After Dr. Vincent left (to do another surgery and then see patients... I don't know how he does it.  He's amazing!) the anesthesiologist came out to talk to me.  She was given a block, aka a kid's version of an epidural, and her pain was managed well.  They did intubate her, which they told me they would before surgery, just because of how long surgery was going to be.  She wanted her to be able to take some deep breaths and felt it would be best for her.  Everything went great as far as that goes.  Deena did have some blood loss, but her blood test came back ok, so she did not need a transfusion.  We will watch her closely for the rest of the day and give her one if needed, but as of right now, she is seeming ok.  The anesthesiologist gave me a hug and then told me they would be out to bring me back.

Waiting is SO hard but I finally got the call that they were grabbing me and then bringing us to a room.  Deena has been sleeping pretty much since she got out of surgery, waking up just long enough to wiggle her toes for them.  She also threw up a little bit, so they gave her zofran.  The plan is to let her sleep as much as she needs and they are doing a good job not disturbing her.  She is on a regular diet so she can eat and drink whenever she is ready, though we will take it slow since she had a tummy ache. 

Thank you for keeping us in your thoughts and prayers.  I'm praying her pain is able to be managed well and she can bounce back quickly.  She's so tough and resilient.  She's pretty darn amazing!

 

Catching up on Posts, but a Prayer Request First

I'm sitting down for the first time in.... months :).  Ok, not really, but sometimes it does feel like that.  I finally have a few moments to update the blog, but first I have a prayer request.

As we speak, Deena is in surgery to rod her right femur and her right tibia.  We knew that this day was coming, but we wanted to wait and let her get some of her PAM infusions before rodding.  When we checked her right femur last, it was evident that the time had come.  They rodded her right femur in India with what we believe is a generic Fassier-Duval rod.  The rod may not have ever been put in correctly, and also did not deploy correctly.  Because of this, the rod is positioned badly and is protruding from the bone.    The bone has also curved around below where the rod has slipped up. 

Deena is in surgery to remove her old rod and put in a brand-name FD rod correctly.  We will also rod her fibia, which is also very curved and weak, since she will be under for her femur, and it will save her a surgery in the future.  A representative from the company that manufactures the FD rod will be in her surgery as well, which is pretty cool.

Dr. Vincent is her orthopedic surgeon here in Tucson.  He used to work at Shriners and has a lot of experience with kiddos with OI.  He's an amazing doctor and we are thankful she is in such good hands!  We also found out he lives in the neighborhood we are renting in, which is fun.  It's nice to know he is so close in case of an emergency.  That's been one thing that has been hard for me.  In Phoenix, I always had people to call if I had an emergency and needed back-up for my other kiddos.  Our rental house is close to David's work, so he is able to get home quickly if we need him, but Sundays are hard.  David plays drums in the worship band at Church twice a month, but our Church changed locations and is now about 35 minutes away.  If we have an emergency on a Sunday morning he plays, I'm not sure what we would do, because he can't make it back home quick enough.  We love our Church, though, and the kids have made a lot of friends there, so we don't want to uproot them again.  Dr. Vincent's wife said if we have an emergency, we can call them and they can be at our house in about 4 minutes.  Knowing that has given me so much peace and for that I am very grateful! 

Anyhow, back to surgery updates.  I woke up this morning at 4 am so that we could get to the hospital at 5:30 for check-in.  I'm not a huge fan of being up before the sun, but we made it!  Dr. Vincent said the surgery should take about 5 hours, so for now we wait.  The tricky part will be getting out her old rod, since we aren't exactly sure what is in there right now and it's positioned incorrectly.  They found an anesthesiologist that let me gown up go back with her and talk to her while she fell asleep.  I love getting to be there while they drift off.  They said they will get me while she is waking up too, so that she wakes up to me being there.  Her anesthesiologist has been wonderful so far and I will definitely be requesting her in the future.

I'll update more once they come and update me.  Thanks for keeping our sweet girl in your prayers!

Poor Deena's Leg

(Another post to catch up)

A few weeks ago sweet Deena broke her leg again.  This time it was her left fibula/ tibula.  Ramya was in a not-so-great mood (company had just left and she doesn't transition well when people come in and out of the house).  She decided to try race Deena, though Deena told her she didn't want to race and wasn't racing herself, and accidently swung around and kicked her in the leg.  It was an accident, but hard enough to break her bones.  Ramya is an all-or-nothing kid, with no middle, and she was in a funky mood, so it's all it took.  Deena's lower leg bones are very curved and need rods, but we were waiting a bit to get a few more PAM infusions in her first.  For now, they are just very, very fragile. 

Deena only cried for about half a minute, and was mostly just yelling at Ramya and calling her mean.  She asked me to splint her leg, so I did, and then she told me she was fine.  I kept noticing little things, though, that made me think she was favoring it.  When I would ask her, she would say she was fine and not in pain.  She was putting partial weight on it, scooting all over the house, bending at the knee, moving her toes around, and playing like normal, but something seemed off.  I called the orthopedic surgeon's office, explained what happened, and they told me to watch her and continue to evaluate her, then decide if she needed to come in.  I continue to watch her and was still stumped, so I called and told them I needed to bring her in.  When the doctor took off the splint he cold tell it was likely broken because she cried and didn't want it touched.  In hindsight, I should have probably done that myself earlier but I'm learning and new to all this and it just didn't occur to me.  She seemed happy and comfortable with the splint, so I left it on.  Anyhow, long story short, she is the VERY proud owner of a glow-in-the dark cast.  I'm hopeful that, with the incentive of a very cool cast, she will be more likely in the future to tell me if she is hurt.  I'm not sure if she's just so tough and she's used to being in pain that the splint helped enough to where she wasn't in pain, if her pain tolerance is just so high she didn't realize she was hurt, or if she didn't want to be taken in so she hid it from me.  She had told me she hurt her legs a few months back in the pool, but when I took her in, it was fine.  She was acting pretty much exactly the same this time, but this time she wasn't fine.  It's still hard for me to read her, which is just really hard.  I'm praying in the future it will be easier to distinguish if she is ok or not. 

Waiting to be seen

Proud of her new cast, enjoying her first strawberry milkshake

An Interesting Trip to the Dentist

(Another post to catch up)

Deena's first trip to the dentist was a success, but was also not at all what I expected.  I knew she had major teeth issues and would need a lot of dental work.  Initially, our holistic dentist was going to see her, but once we went over the extent of her issues, we decided it would be best for her to see a dentist that could perform the initial dental procedures under anesthesia in the hospital.  It can be difficult to find a dentist that is familiar with osteogenisis imperfecta (OI) and Dentinogenesis Imperfecta (DI), which often goes along with OI type 3.  After getting recommendations and calling around, I found a dentist, Dr. Hishaw at Tucson Smiles, who was familiar with both and who felt like she would be able to help Deena.  Deena loved her and did great with  her first x-rays and cleaning. 

Deena does indeed need extensive work and we are currently working with the hospital to book a date for her that also works for her dentist.  The good news is she was diagnosed with DI, so our health insurance may help pay for part of the hospital and anesthesia costs since that may fall under "medical" expenses.  We, unfortunately, do not have dental insurance, so the dentist bills will still be our responsibility to cover, but it will be amazing if insurance helps with the rest.

The surprise of the day came when we looked at Deena's panoramic x-ray.  Apparently, there is only one set of adult teeth left that haven't come in yet.  The adult molars you can already see when she opens her mouth are all she has, other than that one set.  Many of her adult teeth came down in to the wrong spot (notice the adult canines where a molar should be, for one).  We don't know if she was just missing a bunch of baby teeth, if the baby teeth were pulled, or if they fell out really early, but there was nothing to guide her few adult teeth down, so they came down in to the wrong spots in a few places. It looks like she has a lot of teeth, but in reality, she has multiple places where the baby and adult teeth are side-by-side because the baby tooth never came out and the adult tooth came down early, in a different spot than it should have.  

The plan for now is to deal with the decay and cavities in her mouth, leaving as many teeth as we can, even the baby teeth, as long as they are healthy and able to be saved.  Eventually she will need partial dentures to help fill the gaps since bridges and implants aren't an option for her.  I told her she was going to have a million dollar mouth, and she thought that was pretty great!

Deena loved the dentist so much that she came home and practiced finding cavities on Madi.  Who knows, maybe she will be a dentist one day when she's bigger.  She's a smart little cookie and I know she would make a great dentist!

 

Busy, Busy, Busy Bees

As usual, we have been keeping ourselves quite busy.  We've had quite a few appointments and have found lots of fun things to keep us from betting bored.  I'll update with medical first, and then get to the fun stuff.

Medical Updates:

Ramya and Madi had their first appointments with our new orthopedic surgeon, Dr. Vincent.  Both girls are doing really well and nothing big is needed right now.  Ramya is due for a small wheelchair and HKFO (Leg braces that go up to the back) adjustment.  She hasn't grown much, but just enough to need a small tune-up.  Ultimately we think Ramya will be able to use AFOs to walk, but her strength, leg muscles, and confidence isn't quite there yet.  The doctor asked if I would like to put her in AFOs (for therapy) and start using those instead of the HKFOs.  My gut tells me she's not quite ready, though.   She still has some major fears of falling we are working through and she still fatigues very quickly, but I asked if we could go ahead and get AFOs to start working with, but also keep up with her HKFOs for now also, so that we have options with her mobility and therapy. He agreed, and I got her fitted a few weeks back so those should be ready any time now. 

Madi has grown right out of her HKFOs and we have decided to try RGOs (reciprocating gate orthotics).  They will be similar to the HKFOs but a bit heavier and more sturdy.  Because they are heavier they should give her more support and keep the metal from bowing so much.  We chose not to use them when she was little because she was so tiny and we didn't want to add any extra weight to her leg braces, but we are hoping this will actually give her more stability and help her with her walking now that she is bigger.  She also got fitted a few weeks back so we should be getting them pretty soon. We are very lucky that Ron, our favorite orthotics maker at Hangar in Phoenix, has a brother here that owns Hangar in Tucson.  We were able to get in to see him and I know he will do a great job with the girl's orthotics.

Madi has also grown out of her current wheelchair so we will start working on getting her a new one.  She needs something lighter weight that moves with her better, so I'm looking in to different chairs for her.  I would love to get her a Box chair, but insurance does not usually cover them. That doesn't mean I won't fight for it, though ;). 

The kiddos had dentist appointments to get their teeth cleaned.  The dentist just told me she doesn't think she can help Deena, though, because she thinks she will have to go under anesthesia in the hospital for her dental work (she has multiple rotten/broken teeth and her poor mouth is a mess), so we are searching for a new dentist for her.  Dental is hard with OI because it's hard to find a good dentist that has any experience with kiddos with OI.  I have a few calls in, so we shall see.  Ramya's little mouth is a mess too.  She holds food in her mouth, didn't have dental care before coming home, used to grind her teeth really bad when she was mad, had adult teeth come in without loosing the baby teeth, had her 11 year old molars come in when she was 8, and her mouth is very very crowded.  We already pulled the baby teeth since they weren't budging and they were causing her a lot of gum pain (the grown up teeth were pushing forward and the gums were getting pinched between the two sets).  Next up is dealing with a cavity she has, getting her sealants repaired, and getting her in to an orthodontist to see about expanders.  I realllly wish we had dental insurance right about now.

Ramya started vision therapy, so now both Ramya and Conner are in vision therapy on Wendesdays.  Conner finished his first 8 weeks and has made huge strides, but still is reversing a lot of his letters.  We start a new 8-week round with him this week.  Ramya needs intensive one-on-one therapy, versus Conner's group therapy, because having a lack of visual stimulation when she was tiny lead to eye muscles not coordinating and working together correctly.   Her homework right now is to watch a show on the computer using special red and green filters and special glasses.  When she's having a hard day, I put on a calming video with calming music from youtube and it not only helps her with her emotions but it also helps her eyes, so it works out great.  I have to say, I also really wish we had vision insurance. 

Tonight Preferred Homecare delivered the medication and supplies for Deena's first PAM infusion at home.  She will get them quarterly to help with her bone density.  We are so so excited to see her bones get stronger!  Her first infusion was supposed to be tomorrow but they had staffing issues, so it is now on Thursday.  I'm praying they get the IV in quickly, on the first try, and that she doesn't have any major side effects from the infusion. 

The Fun Stuff....

Now for the fun stuff!!  We tried wheelchair tennis with JAWS and the kids really loved it!  Soon they will change to wheelchair basketball and the kids are excited about that too.  Med students from UofA come and play with the kids, and siblings are allowed to use chairs and play too if they would like.  Conner was in Heaven and had so much fun using a sports wheelchair too. 

We had friends from Sweden visiting in the US and they were able to make a stop in Tucson to meet with us.  Their son, Arven, was in Ramya's second orphanage with her (Ashraya).  Though Ramya does not remember her time in that orphanage very much and did not remember Arven, it meant so much to her to have that connection and get to see him.   Ramya moved so often between being in 3 orphanages and in and out of the hospital during her time in India.  I'm not sure if she just doesn't remember a lot of what happened, or if she has blocked it out, but I know she really longs for connections.  She doesn't remember her friend's names from India and doesn't remember her caretakers, even when we show her photos.  She will tell me she misses her friends, and of course I tell her that it's ok to miss her friends and that I know they were very special to her.  She can't recall any of their names, though, and will tell me the names of friends she has heard Deena talk about, but ones I know for a fact she never met.  Having special visitors was just what she needed!  Now she has a friend she can talk about and remember.  Arven and Conner hit it off right away and Arven ended up staying the night even though the rest of the family stayed in a nearby hotel.  The boys had a blast playing Minecraft together and have decided that we will meet up in two years in Disneyland for more fun.

One of David's brothers and his wife were in Phoenix for 4th of July weekend.  We decided to make a trip down to Phoenix to visit and had a lot of fun swimming and BBQing with family.  We had a fire pit (oh man was it HOT!!!) and Deena enjoyed her first s'more.  Deena also loved her first fireworks and is still talking about it.

Other than that, we are doing some light homeschool activities this summer but are not doing our full curriculum.  The kids have been enjoying extra crafts and science experiments.  We have also been swimming a lot and are happy to have a pool at our rental house.  Deena is like a little fish and has really taken to the water!

(our light up fairy jars)

(bouncing bubbles from our Sick Science kit)

(pendulum painting from Kiwi Crate)

One a personal note...

On a more personal note, it hit me yesterday that I miss having my friends close.  A while back I realized that I wasn't invited to many girl functions any more.  Between our unpredictable schedule, just being plain tired and not always feeling up to socializing, that fact that I stink at initiating things when we are busy, and the fact that I never feel comfortable going very far from home (in case I need to get back quickly for an emergency), I realized I said "no" to a lot of things, and soon wasn't on the invite list.  It was a hard realization to come to (I wrote a post about it if you would like to read it), but it made me even more thankful for the amazing friends that we have who are always there for us.  We were blessed with friends we could invite over last minute, even if our house wasn't perfect.  We were blessed with friends who brought us meals after hospital stays.  We had some really close friends that eat like us, also homeschooled, and had kiddos that our kiddos loved to play with, that we saw often.  I had girlfriends that God had gifted the ability to know when I just needed to talk, needed coffee, or needed a hug, and would always call or show up at just the right time, without ever being asked.  I had some close girlfriends who also had kids with special needs and who totally got it.  We had an amazing Church and homeschool community we were plugged in to as well.   Though I'm so thankful to still be able to talk to my friends on the phone and to visit with them when we go back to Phoenix, I'm starting to feel the distance between us.  I don't have any friends that live close any more, so I have no one to go out with for girl time.  I know I will get there and just need to give it more time.  I was so blessed, and am still blessed, with some amazing relationships and it will always mean so much to me.  We have started to make connections with other families and have enjoyed playdates and social events, so we are thankful for that, and it definitely helps.  We also found a Church we really like, so that's been great.  I'm also thankful for Social Media because it helps me feel connected.  Oh, and I also was given a really sweet letter and giftcards from a neighbor we lived next to for many years growing up.  God knew I needed a little encouragement, and it really meant so much.  A little kindness can truly make a big impact in someone life!

  

I think that's about all I have for now!  Please be praying for Deena's upcoming infusion and I will try to update in a more timely manner about it.  Thank you for following along in our journey and for all of your love and support!

Appointments, Visitors, and Fun

I am behind on blogging, as usual, but I'm trying to be consistent ;).  Ok, not really, I just operate slightly behind, and thankfully it doesn't bother me too much.

A few weeks back we attended an event for the Tucson chapter of the Arizona Spina Bifida Association at a local gymnastics gym.  The kids had a LOT of fun.  I plan to get them on the wait list for future classes.  An OT leads frequent classes for kiddos with disabilities and is really great with the girls.  Conner has been wanting to take gymnastics too, so it works out great!

 Conner got to take his turn and have an appointment with a vision specialist.  He was pretty excited actually.  I had all the kiddos evaluated in Phoenix, just to make sure that not only their vision was fine, but also their tracking and the such, and Conner showed some gray areas where the doctor thought he may eventually need some vision therapy.  His eyes have a hard time converging when he's reading.  It's not major, but seems to be effecting his reading a bit.  I have noticed as the print has gotten smaller, he closes one eye when he reads, sometimes skips lines, and still reverses some letters. I took him to get evaluated again at a different place in Tucson an they decided that he would benefit from glasses to magnify his work whenever he is doing school work or watching TV.  He also is going to do 8 sessions of vision therapy.  He is super excited for his glasses and very happily wears them.

Madi's new neurologist wanted to do a sleep deprived EEG to see how she is doing and possibly switch up her medication since she's still having a decent amount of seizures, and her seizures are hard to stop.  He wanted me to give her about half the sleep she normally gets.  At first she thought staying up and watching movies with me sounded amazing, but she is my girl who loves her sleep, and the novelty wore off quickly.  She did it, though, and I was very proud of her! She did well during the EEG and had the nurses all giggling, of course.  The doctor said her patterns have remained the same.  She has a lot of mis-firings, most stemming from the left-hand side.  We discussed new medication for her and I told him my concerns with picking a new one.  She only has one functioning kidney, so it's very important to have her on something that will not cause any kidney damage.  She is also terrified of blood draws, so I wanted her on something that did not require frequent level checks.  We decided to try lamictal since it works well with focal seizures but also helps generalized seizures.  There is a risk she may have a rash/reaction, so we are taking our time and getting her up to the full dosage very slowly, over a span of 4 weeks.  At that time we will see how she is doing and hopefully slowly wean her off the Keppra.  The keppra makes her moody, so I am hoping the lamictal helps her there, though I'll take a moody girl with a happy kidney over anything that may harm her kidneys any day!  After the EEG I took her out to eat and she chose Panda Express since we do not usually eat there (gluten sensitivities) .  She was pretty excited!  I also got a coffee (because momma was sleep deprived too) and she convinced the barista to give her a cup of whip cream with chocolate on top.  This is a rare treat for her (due to her dairy sensitivities), plus they gave her a huge cup, so she was super excited! 

(She looks grumpy but really wasn't.  She was in a good mood but was just tired)

Last week we had visitors and really enjoyed our time with them!  Our friends Darlene and Samuel from homeschool co-op in Phoenix came by to visit on their way to see family.  They brought us a pretty lavender plant and some gluten-free yummies and the kids had a lot of fun playing.  It means a lot to the kiddos to see a familiar face!  My parents also came in for a night for Mother's Day.  We had planned to stay in Tucson since we have to be in Phoenix again at the end of May, so they decided to come here.  They hadn't been here since the end of last year and the kids were really excited they visited.  

Deena had her follow up with the orthopedic surgeon and everything looks great!  Her rod is in perfectly and her bone is healing nicely.  He said if she felt good she could take off her splint, which made her VERY excited.  We kept it to put back on if she has any pain, but so far she says she feels great.  I'm trying to keep her going a little bit slower than normal while it continues to heal, but she's a feisty little cutie and not always great at that.  

Other than that, the girls are liking our new occupational therapist.  She seems pretty proactive and works well with the girls.  This week she brought fun dress up clothes to practice dressing and the girls loved it!  I think I have speech therapy lined up as well, though finding physical therapy is proving to be a challenge.

Oh, and this morning we woke up to cookies at the front door from my dad (AKA papa) and then found that all our butterflies had emerged from their chrysalises so we got to release them.  The kiddos were pretty happy!  David got me an in-home massage for Mother's Day and she came today, so I had a pretty good day too.  Tomorrow David's brother, Mark, and his family are coming to Tucson for a visit.  We are really looking forward to spending time with them and are excited for more visitors. 

I think that's all for now!  I hope everyone has an amazing weekend!