3 Long Months of Isolation Broken!

Thank you God! For +15 weather in January.

Oct. 15th, 2014 was the last time our Bella got to be outside, outside a hospital room to be exact. Since then she has been on protective isolation and yesterday we got a half hour of heaven outside in the sunshine. 

Be prepared for some amazingly cute Bella Bombs! 

Since being on isolation to protect Bella, even going down a floor to a clinic for any pre BMT work up was tricky. We are obviously not aloud to walk her around anywhere in public. So twice since October she has needed to go outside her hospital room and to do that they use an isolet. She absolutely loves these trips and she received so many smiles, and "awwws" from all the people in the hallway. 

When our doctor saw how much of a joy this was for Bella she wanted us to take advantage of the beautiful weather and allow Bella to get some fresh air outside where there are no bugs. So we jumped on that chance yesterday and Bella loved every minute. 

We put a mask on her to walk down the short hallway in the unit, to the back door that only unit 1 nursing staff go through.  

From there we got to explore in the little garden/walking path area outside Bella's room, her bunnies home! We looked for the bunnies, but they weren't around today. Lyle took her for some fun scoots in the highchair, and then we walked around exploring trees, bushes, rocks and more. 

It was, shall we say, 'A breath of fresh air' to see the joy on her face. 

Today was the second ride in an isolet down to the dental clinic. Bella had her first dental visit today and her 5 teeth look beautiful. Her gums and teeth are very clean and healthy. Mainly due to the fact we have not been allowed to feed her and therefore her gums and teeth have not been exposed to much. None the less she has a super healthy mouth! 

Fun Times This Week

So Bella's Unit 1 room is ground level and is beside a beautiful garden area with trees, bushes, walking bridge and basically a cute little spot for a pair of bunnies to hang out. So we named them. Gus Gus is right beside Bella's window every morning, so they have tea together! Stinker is the bigger one, and hops around all over eating many twigs n bushes. 

Need I explain that picture? Haha 

Music Therapy. 

Unit 1 has Musicians come in and play instruments and sing to patients. They also have the patients partake in making music! Bella loves music therapy time. She shakes the maracas and pats the drums! She also loves the chimes and touching the guitar strings. She lights up every time.

SLP/ Language/Games

Bella is a ham and she is always so happy. She initiates her own games and the SLP is very impressed with her expressive and receptive language. Bella is advanced in these areas for her age! Yay! Love that report. The SLP said she doesn't work with kids that are advanced, she works with kids to catch them up. But she told me she was so intrigued with how advanced Bella is that she wants to work with Bella and I to keep up her advanced level. 

Bella's words:

Momma, daddy, dad, dada, Bye bye, baba, all done ("aah- da"), grandpa ("papa"), grandma ("bah-ma"), balloon ("baa-oon"), Apple ("ap-oh")

And many other sounds and babbles. 

Bella's sign language/motions: 

She signs for: more, up, pointing for 'wants', down, waiting, waves hi n bye, dances to music in many ways, claps, blows kisses. I love catching her dancing to the music when her favorite cartoons come on. 

Games: 

Bella's favorite is initating peekaboo. She plays peekaboo behind whatever is available like a book she's holding, her own hands, crib rail or even her blanky corner. She loves to copy cat with blinking, clapping, stepping, jumping, smacking lips, and making funny "chin up" faces. Oh and she loves that Big Bird (the puppet) takes her soosoo, so when she sees him she offers her soother... But only to Big Bird. Oh, and moms tickle fingers get her so excited. She is a tickelish girl! 

Time Is Of The Essence

Time... 

That word means a lot for Bella right now. So much needs to come together in the right way for an "Urgent" BMT case such as Bella. 

Waiting Time: In the time we wait, Bella can not get sick in any way that could compromise her getting the BMT. 

10/10 Comfirmation: Currently we are waiting for the 10/10 donor blood work to be confirmed a 10/10. We are also waiting on the back up 9/10 to be confirmed useable for Bella. In the last post I mentioned finding out if the team continues to look for more 10/10's or not. 

Simply, no. No Time. We need to use the matches that have come in so far. Bella's need for an urgent BMT means using the first chance at a 10/10, or the next best 9/10. Her case does not allow the time to wait for another 10/10 if our current one doesn't work. That's why it was so so hard to hear that the first two10/10's were no longer available. This current 10/10 match that is being confirmed is it! Bella needs it so badly. 

Bella doesn't have the TIME. Her need for a BMT is now, urgent. And her body needs to be at its best and strongest going into conditioning (chemo) and transplant so she has the best possible chance of accepting the donor marrow and no rejection. 

Therefore we are praying and believig this current 10/10 is it and available for Bella. Otherwise the team uses the next best live donor 9/10. (Still posses more risk than a 10/10.) What our team has gotten back so far is another three 9/10 matches. So we have quite a few 9/10 backups. But we want the 10/10, it's best. However, the BMT team knows they still have 3 registries left to respond to the donor search, that could still have 10/10 matches, but they don't have the time (weeks/months) to look into those. Bella needs a BMT now.  

Either way she has a 10/10 now and as long as that perfect match is confirmed in the next week, we use it. 

If not, they would go to using the 9/10. 

If this 10/10 were to for some reason not be confirmed it would be too risky to take the time to look for more 10/10 matches. Too risky to allow more time and chances for Bella to get sick which would mean risk of not getting the BMT. And that can NOT happen. She needs it asap to fight infections and have a working immune system.

POP the First Bottle of Champagne

Lyle and I are going to pop our first bottle tonight and celebrate the good news for Bella, but there will be a few more Crystal pop'n moments before we get to the big bottle of Baby Duck! 

So what we found out today is that the perfect match (10 on 10) donor has been contacted and gave their blood sample to send to Calgary and it should arrive in Calgary today! Calgary's lab will receive it and test it to confirm the 10 on 10 match. From the time that Calgary lab receives the blood sample till they are done testing the matching, may take a 1-2 weeks. 

This is truely amazing news because this means the donor has already been contacted and agreed to give the blood sample and its sent! :)

Bella needs a back up donor and we also have that. So currently a 9 on 10 match donor has also been contacted and sent their blood sample to Calgary as well. 

I still need to meet again with our BMT nurse and just ask her if they continue to look for a 10 on 10 back up donor aswell. I'm not sure about that yet. 

Here's to us! Cheers for Bella! Let the Celebrations begin! This may be just the beginning but I believe in getting your hopes up, I always have because I have HOPE and FAITH and I'm not letting go of that ever, for my baby girl. :) 

Info Overload (BMT update)

I've already starred at this screen, preparing to write this post for the last half hour trying to fathom how I can possibly try and explain to you all the thousands of details I've taken in lately... *sigh
I just need to start writing. I'll spit it out as best I can, but the words and phrases are running through my mind and then leaving it, faster than lighting. I need to trust that those of you who read this understand that there are many more details I can't describe in words, the moments and pauses Lyle and I had in the meeting where the info we received made our hearts stop...and then beat again. Please understand I want all of you to clearly understand what our baby girl has to endure, and its hard to put emotion into a keyboard, but the info I have taken in has been scary as hell but parts have also been so extremely hopeful, happy and rare!

The Big News
Our BMT team was amazed that within the first couple weeks of their search, three 10on10 (perfect matches to Bella) came back! For most it takes months to find even one 10on10 match. That is truly amazing, giving that OneMatch is closed for xmas holidays as well. They started the search Dec. 18th, 2014.
Bella needs a perfect 10on10 match for her BMT, that gives her the best possible chance of her body accepting the donor's marrow and least possible chance of rejection...even a 9on10 match is much more risk for Bella. We want a 10on10, Rejection and complications can mean death, we want the least possible chance of even thinking we may lose our baby girl through this.

Here's the gut wrenching, punch in the gut... 2 of those perfect matches are no longer available to donate. They can't tell us why, and trust me, our BMT team digs and digs to make sure...but they just can't. Perhaps they became ill themselves or something serious came up, needless to say they can't be Bella's donor.
HOPE: One out of the 3 that's left, they (Calgary) are still waiting to hear from and we are praying they are available to donate. The process allows them to only look for 3 donors at a time.
So why would they even tell us? Right? Well, because they are so so amazed at finding 3 perfect matches in the first shot that they want us to remain hopeful that Bella is a great candidate to find and be able to rely on another perfect match.

So Now What?  Here's What We Do:
We keep Bella at her absolute best before transplant. We want absolutely no chance of anything causing this transplant to be put on hold, Bella needs it ASAP, and she needs to be at her absolute best going in. Currently she is on meds to suppress her immune system, keep her skin under control (reduce flare ups) and watch her closely to make sure no chance of any virus or infection comes near her.

Time Line
1. Currently: Waiting and completing Bella's entire pre-transplant work up. This is where every doctor in the hospital checks Bella over to gather all her 'normals' and baselines, pre-transplant. I laughed when they told me "every doctor in the hospital"...been there...still doing that. Anyway, these work-ups will be on everything from Bella's dental check to a full CT scan.

2.Wait: Pray for an available 10on10 match

3. Confirm A Matching Donor:  Once a donor has been found and confirmed they go through rigorous rounds of testing and evaluations. (1-2 weeks) The final day before Bella starts her Chemo is the day where the donor makes the absolute decision to say, "yes" or "no" to donating their marrow. They have a counselling session this day. When they have said, "yes" at this session, Bella begins her Chemo.

4. Day -8: This is the start of Bella's Chemo. 8 days of Chemo meds to prepare Bella for her BMT. These may be part of the hardest days through this process. This week they kill Bella's entire immune system in a very specific way so that there is the best possible chance her system will accept the donor's through transplant with no rejection or complications.

5. Day Zero: Transplant Day. Bella receives the donor's bone marrow through her IV line. I probably will still have not slept for the last 9 days at the point.

6. Post Transplant Days: Every day from here on out is a plus +.  From +1 day and forward they monitor Bella very closely to make sure of no rejection or scary complications while her new immune system builds. For the first couple of weeks we still have to be so so careful and pray and pray that no complications or risks become serious, or happen at all. Each day she is well and gets better is going to be a celebration.

7. Day 100:  This is the day (post transplant) that they title Bella to be "well-engraft" or in other words, "out-of-the-woods."  So that's about 3 months in hospital monitoring....we still have at least 3 more to go.

8. Dropping Meds:  After Bella is well-engraft and if she continues to do well they still have certain monitoring times-lines for kids post transplant. They usually say to expect to remain in hospital 6 months post transplant. So after Day 100 and for the months after that, they will look at how well Bella is doing and when the time is right, start taking her off all meds. Not only is a BMT a cure for Bella, but this process means she does not need life long medication. So eventually she will be off all meds, and not rely on any meds to remain healthy, her new immune system will do that work!

Now you think I have covered it all...no no no my friend. I haven't even started telling you about the Genetics side of this whole story. We know Bella has a Immune Deficiency, one so bad that only a BMT can cure...but we do not know why. Why Does she have an Immune Deficiency?
That info is for another day, I'm beat.

Long Day, Unit 1 Move & Big Day Tomorrow.

Well, it's about 10pm and after a quick first coffee at 12pm today and a chance to grab a hard boiled egg for a snack (breakfast, lunch and supper... thank God for that egg) I just had a chance to eat a meal. Same with Lyle, he's been work'n his butt off today and Bella's been a trooper through today as well. 

Medical Update

Today Bella had her monthly IVIG transfusion (blood product). This gives her an antibody boost. So she's hooked up to so many wires and lines for 3 hours I can barely change her diaper. And because it's giving her someone else's blood product, nurses had to check her vitals every 15min.

She also had her 3rd RSV shot to help protect her from serious respiratory illnesses. She also had to deal with a bandage dressing change this morning that took too long. (Covers her iv line in her chest) She has to be held down for this and is really upsetting for her. She's so smart, she gets upset when she sees the nurse gets all the supplies ready. It's not supposed to take more than a couple minutes because if it does, Bella almost has panic attacks and gets really worked up and is then really hard to calm down. Im praying these bandages last longer than 3 days at a time, I don't want her to go through this every time the bandage covering her line needs a change, for the next however many months. :(

Meds:

Erythromicine: She's done with this one! 2 days so far and she's poopn great. She has been on this med since July 2014 for motility of her bowels and is now completely off it. She's adjusting well, a lil pukey after a feed but poop'n great. So her bowels shouldn't need help from this drug anymore! 

Ranitidine: she gets this twice a day to help keep her tummy happy. And it also helps reduce acid in her tummy. 

Septra and Fluconisol: These are antibiotics given as a profolactic Med right now. They faught the infection in her lungs and esophagus and now are at a preventative dose to keep Bella at her best before BMT.

Prendisone: this is a large spectrum immune supresent med/ steroid. Helping control Bella's immune system. Even tho it's not much of an immune system it's not doing what it's supposed so it's causing problems like her harsh skin flare ups. 

Sycosporn: more direct immune supresent med to help Bella be at her absolute best before BMT. Bella will just be starting this med tomorw. 

UNIT 1 !

Part of today being so long is because we moved units today. We are officially in the oncology unit now and Bella has a new room. So we said "Good-Bye" to unit 2 today. We'll miss all of our nurses that knew Bella the best. But we've heard so many wonderful things about the very specialized nurses and team of Unit 1 and we are looking forward to getting to know them, and for them to know our Bella. 

Tomorrow: 

We have our much anticipated, big meeting with the BMT team at 10am. They generally don't have these meetings with the family until they have, or are very close to having, a match! So we are anticipating big news tomrw, but who knows. Hoping and praying!

The Past Week

The holidays were great and Bella's doing really well. We are still in a 'waiting' mode for a lot of Bella's results for many things. We have a big meeting with Bella's Bone Marrow Transplant team doctors this Wednesday. We will have more updates then. 

Bella had a wonderful Christmas! This year (Being 1y old) we saw her sheer excitement in discovering what it is like to rip open gifts and find out what's inside. She would rip the wrapping paper with this hilarious "shake" and just loved it. Last year in the NICU in Sasktoon, we had a much more quiet Christmas. This year, though still in hospital, Bella had so much fun. 

TEST Confirmed:

Bella's Immunologist knew from the start Bella had SCID, she knew she has had an immune deficiency from birth, but the question was how severe? Moderate or Severe? So after she had Calgary lab do all the tests, we found Bella's immune system operates at 4% of normal. So basically no immune system, so very severe. Hence why she is on the "urgent" list for a BMT donor. 

Our Immunologist sends this same lab work to a Gold Standard lab in Cincinnati to confirm Calgary's results are accurate. And so we just received that info this past week. And yes, they confirmed Bella's immune system operates at 2% of normal. So yes, it is confirmed that this girl needs a BMT.

It's technically only been 2 weeks since we stared the search for a donor, as Lyle and I are not a match for our baby girl. However we are told Bella has real real good chances at finding a donor in the international data base, and we can't wait to thank that person with every bit of our hearts, for the rest of our lives. We are praying and believing that match person for Bella will be found soon. 

Again, we'll have more info on this after our meeting this week. 

In the mean time, Bella still has her weekly blood tests, monitoring, physio, etc on Unit 2. Things are going very smoothly right now (minus the ups and downs of teething) but that's a "normal problem"... we welcome those. :) Her purly whites are sure maken that smile cuter and cuter!