Just Relax, Figure It Out, Try Again. Day +357

Today is Day +357 Post BMT (Bone Marrow Transplant. Yes! Her 1 year post transplant day is coming, her Re-Birthday will be March 25th 2016.

Today is also Day 95 since we have been out of hospital and at home.

Those words in this post's title are what I have found myself needing to say a lot to Bella lately, when she gets frustrated and sort of "Hulks Out" like I do when things don't work out. I need to start taking my own words of advice I give to my daughter, and try them for myself. Apart from this month going exceptionally well for Bella, I've had some of my own struggles weighing on me. But, we'll get to that later. For now:

Just Relax, Figure it Out...Try Again. 

Bella's Progress >> Gravity Feeds

This big step towards being backpack and feeding pump free is a huge step that daddy helped Bella with. Bella and her bowels have improved a lot and are not as sensitive as they once were, so now she can take in her needed fluids with quick gravity feeds instead of having to be hooked up to a feeding pump. The pictures above show me accessing Bella's Mickey Gtube button to hook up a  short feeding tube to and then pouring in her needed fluids. (Formula, water or Pedialyte) This (unlike the pump) mimics more of the natural way we take in fluids like drinking a large glass of water by mouth instead of sipping on it for 20-30minutes. Bella's gravity feeds are going so well because she really only requires "top up" liquids because she is eating all of her calories by mouth. Because Bella can't gulp down her required 800ml of fluid in a day, being able to top up her fluids with gravity feeds this way is very freeing. Bella's drinking skills have improved but she is currently only able to drink about half a cup of water during each meal. (sometimes less) Bella requires at least 400ml of water and 400ml of Pedialyte in a day when she eats ALL of her calories that trump her formula need. This of course is an excellent accomplishment for Bella, eating so well. Her dietician is so happy with her eating a large variety of foods as well. Veggies, fruits, grains and meat....she loves many kinds of all of them. When Bella eats this well, it saves us a lot from the huge formula cost. But she's not at an age where I can reason with her yet and explain to her she must drink more water than the average kid because she has no colon and her current medications require so much water. So for now we are more than happy with her victory of tolerating gravity feeds, and we'll work on helping her drink more water by mouth when she's ready. 

Our last Calgary visit

Hotel, Clinic, Surgery

Those files in the pic are just a portion of all of Bella's ACH (Alberta Childrens Hospital) files and don't include her files from RUH (Saskatoon hospital). They have to bring them out at every surgery. 

I have SO many updates from Bella's last doctor visit in Calgary! I'm happy to finally get to this blog post, but we have been very busy at home and when I don't get any breaks because of what Bella requires medically, and hubby works so one income can support us right now... this blog gets put on the last of my to-do list. However, I need time for this, time to share. I need the support of others knowing what we go through and understanding. So thank you again for following, reading, loving and understanding. 

Ok, back to updates from last Calgary visit. March 1st & 2nd:

Adios Noodles

Aloha Pokes

I posted a video on Bella's Facebook page showing how well she came out of recovery. You can check out her video and more updates not noted on this blog, by going to her Facebook page: Isabella's Mustard Seed.

Click this link to get there:

https://m.facebook.com/Isabellas-Mustard-Seed-848573408556136/

Bella had surgery to take out her Broviak. These lines (or noodles as we like to call them) saved Bella from hundreds of pokes. She had this Broviak (I think the 6th she's ever had put in) for 1 year. These lines are meant to be in patients long term, that is true, even longer than a year can be ok. But for Bella, she is progressing, we are blessed in many ways that she is progressing so now in her case these lines posed more of a risk to her than a benefit. So they had to come out. 

Bella's first year in hospital and all the complications that came with it caused 3 Broviaks (6 surgeries/to put in and remove) and countless pokes to Bella in between those first Broviak surgeries. I wanted so so bad to never go back to that. I remember her being such a hard poke, and not just your average hard poke, I'm talking "Calling the PICU and NICU and Transport Team nurses and they still couldn't get it, so call the Anesthetist with the ultrasound machine to find a vein on Bella guy" hard poke. 

Ya... That hard of a poke.

I remember one of the hardest days ever (besides her Septic Shock days) was where they poked her 21 times. This was horror. And I hate myself for not being more of an advocate for her at this time but I did not know then, what I know now, to save her from that horrible experience. I didn't know we could call the NICU nurses, or try the freezing cream, or hold my ground enough to make them give her a break. Instead she was barely 2 months old, with bowels that didn't work and so dehydrated that if they did not get that IV in her that day at that time she would die. So they tried her hands, her feet, her elbows, her arms and finally her head. And if ever a day is harder to explain then that day.... Seeing her shake from the shock of so many pokes to try and save her life...I have no more words for that sort of pain. 

And so when I come to this point of trying to see this "poke saving Broviak" removed as a step forward... It's still extremely hard to see it as a good thing. 

So, Relax, Figure it Out, Try Again

And from there I came up with this for Bella to help her through her future pokes:

 All Done Pokes Kit

I was not about to let Bella's tears from pokes bring back all those horrible memories from her having to be poked before. I of course want to do everything in my power to keep her strong, brave and able to get through this. Having the luxury of a Central line in Bella for a solid year made me forget all the tips and tricks that help one deal with a poke for bloodwork. I did not want it to take even ONE single poke before I realized, "Oh, this would of helped, or that." 

With the support of many other parents in my Facebook groups I wrote down a list of supplies and ideas to remember for any of Bella's future pokes. And then of course the toy kit itself, to help end those tears after the poke and help her know she can get through this without worry or stress. 

My list of ideas is maybe hard to see in the picture so I'll type it out. Please share with any other parent you know that may have a child needing a poke:

>> Be well hydrated, request numbing cream on the site, wear long sleeves so the blue band they tie on doesn't pinch the skin, wear jacket and mittens prior, to heat the vessels to make it easier for the nurse to find the vein. If the poke is in the heal or foot, wear 2 pairs of socks. Hold heating pads or put hand warmers in the mittens/socks. Distraction ideas: iPad, ring a bell right before the poke, have a treasure box of toys ready, party blowers or funky whistles. <<

So now I make sure to carry this kit with me for every trip to the lab she must endure from here on out. And as hard as it is, we are going to hope Bella never requires another Broviak again, because that would mean things went wrong. 

As I mentioned in the previous blog post, part of Bella getting her Broviak out meant her new immune system is doing better and better (and it is) and that's supposed to mean bloodwork(as in pokes) should only be needed once a month now. Well, I'm not impressed with how the first month with no Broviak is going. One poke in a month has turned into 7 pokes. Her first week after she was home from surgery involved a lab error when reporting Bella's potassium level and that one poke turned into 3 pokes in 5 days. She has more blood work coming on Monday and then praying for a 2 week break only to look forward to 3 pokes needed in 3 days when we are back in Calgary. 

Why Does She Need 3 Pokes in 3 Days?

Part of the reasoning is good news...Bella's B cells are back! We received the good news today. The med that fixed her Hemalytic Anemia knocked out all of her Bcells. Which, by the way, was extremely bittersweet after celebrating a successful Bone MArrow Transplant only to have the important Bcells that we prayed so hard to come in and function with the Tcells to make Bella's antibodies...be taken away on purpose. 

Then we waited through the set back of 6 months and finally found out today that they went from 0-93%! So now that her B cells are back her Immunologist can start a blood test that we haven't done yet, the very important function test. This type of blood test can only happen on a Thursday and Bella's regular clinic bloodwork can only happen on a Tuesday. Also, the blood draw for all these tests would be way past her daily limit, so it has to be 2 pokes for bloodwork. So what's the 3rd poke? Well, Bella still requires her monthly IVIG booster infusion and RSV infusion and you need an IV for that. So after they poke her Tuesday morning for bloodwork, they will have to poke her an hour later to get an IV. I asked why they can't just use the same site and save her a poke. It is too risky to ruin the vein needed for the IV with the draw for blood. So they need to use a different site, a 2nd poke. 

We are looking forward to Bella's special Immunology bloodwork that will be done on Thursday, April 7th. This is a special test of her immune system that they haven't ever done before and are only able to do now, now that she is off all steroids, immune suppressants and her Bcells are back! 

What we are hoping and praying for:

We want Bella's T cells and Bcells to remain 100% donor and function together. We want the next tests to show that Bella is making her own IGGs, her own boosters so that she no longer requires the IVIG boosters and pokes for these boosters. We hope and pray her Bcells function perfectly with her Tcells and remember invader cells so that she makes her own antibodies and THEN we can go on to get her immunized/vaccinated! 

So you can see, so much to look forward to with this new upcoming blood test/immune system tests. If this all works out it means Bella will no longer require monthly pokes for IVIG infusions and our world of isolation will be over once she is able to be fully vaccinated. That's HUGE. 

Bella's First Line Free Bath

No I didn't get a picture of this, it was too exciting (and wet) to enjoy. The freedom of not having to worry about infection in those lines is priceless. I sort of stood there stunned for a moment, elated that I no longer have to Saranwrap and tape her entire chest to protect those lines, before a bath. They're gone. Bella splashed so much that I think she may have drank most of her bath water. I pretty much had to keep her from diving in, face first because she was so happy with her freedom of splashing and enjoying a tub full of water instead of a puddle for a sponge bath. It's been great. 

Other News From Our Last Calgary Visit

When Bella goes to the BMT clinic now, at ACH she no longer needs to go into ISO (isolation) rooms. Because her immune system is doing so well we now go to the common area for her doctor visits and infusions. Now that Bella no longer has her Broviak she will go to the lab on the 3rd floor for bloodwork. Small steps, but steps in the right direction and away from those lonely, difficult extremely isolated days. Now she can go to clinic and maybe sit beside a friend with a smile, or play with another little one in the waiting room. This doesn't mean I'm allowed to take her to just any waiting room, we still must protect her from busy public areas, but the BMT clinic is different. And if Bella is ever sick when she arrives for an appointment, we would be put back in an ISO room. 

I asked some more questions around what Bella is and is not allowed now that she can be around healthy friends and children. Bella is allowed to play with friends now but her docs suggest no more than 6 kids at a time. A lot of this precaution now is because Bella is still immune compromised as her new immune system needs the best possible chance to advance and get to the point of absolute normal function! And she needs this current protection to remain in place until she is fully vaccinated and then that can protect her even more. Anyone she is around must have had their flu shot. Any kids she is around that are older than 1y must have all their shots/vaccines/immunizations. And she is allowed to play with children under 1y if they are getting their immunizations. 

I also asked about Bella being allowed tap water vs the distilled/sterile water she has been given since before transplant. Her doctors are not allowing her tap water until she is fully vaccinated, as a precaution. So we continue to buy large jugs of distilled water for her. 

Sadly, I was told, "No" to starting her in swimming lessons now that her Broviak is out. The reason is mainly to do with her ear tubes. Bella had ear tubes put in during one of her surgeries before her transplant as another protection against any possible infection before BMT. The ear tubes would allow draining, to prevent build up and infection inside the ear. You must wear a head cap or ear plugs to swim with ear tubes, or have them removed. So Bella's ENT Doctor would like to take another look at her ear tubes and ears before we allow her to swim or have her ears go under water in the bath.

It was a long 2 year wait but Bella has finally had some wonderful chances to play with her healthy friends. It has been such a joy to have the support and understanding of very close friends to make these days happen for Bella...and me. Bella and I need these breaks outside the house very badly. And as much as I thought I would be overly nervous about Bella touching toys I haven't bought or cleaned...I wasn't. I have such supportive friends that they reassure me their child's toys are sanitized, bleached and re-cleaned just so I can be a little more relaxed and less on edge as we take on yet another life transition from hospital life to home life. Thank You. 

We are looking forward to Easter break when Bella will have more play dates with her many cousins that don't live in Swift Current. 

From the baby that keeps her mask on to the girl who keeps her bows in.

Bella is doing wonderful and I think the last month at home has been her best yet. I've really seen a shift in her personality since she had to be the kind of kid that tolerates living in a hospital to becoming a regular toddler understanding home life and friends. I've also seen a shift in her patience. I used to have so many nurses, friends, people comment on how patient Bella is for her age. How good she was at tolerating everything from being still for bandages and line care to a doctor poking and checking her all over. And I knew that came from her unique start to life of living in hospital for 2 years. I hope that her tolerance and patients stay with her, but I'm starting to see the effects of home life shift her to what you may call "normal toddler ways" or maybe even "the terrible twos."  I hate those preconceived titles for stages that average kids go through. As much as I want Bella to experience the joys of "normal life," I'm never going to call her "terrible" or tolerate her tantrums just because she's a certain age. Yes, she has had a very unique up-brining so far, but I am still go to try my hardest to keep her experiences unique to her advantages. After what her and I have been through, I don't want to expect the "terrible twos" I want to continue doing my best to teach her patience and compassion, empathy and love. God knows there are enough people in this world that only care about themselves. No matter what age Bella is, I really want her unique start to life to help her understand how much compassion this world still needs.

Swift Current SLP, OT and PT

(Speech, Occupational and Physio Therapy)

We finally were able to meet our Swift Current team of support that Bella's Calgary team transferred her to. I am happy to announce we don't really need them! That sounds odd, but it's a good thing. Once they were able to meet Bella and compete their assessments on her, it was very clear that they are here for support when she needs it. What that all means is Bella is of course at and above age level for all her expressive and receptive language. Physically she is where she needs to be and actually further ahead then expected given the timing of all her major bowel surgeries and BMT. So for example her walking and physical skills are great, her gross motor skills are on point. Side note, she's a walking machine these days, no crawling or sitting...all standing and walking aaaand running. Ha ha. Especially when I tell her to, "Come here, I gotta change your bum!"...she scurries away with a giggle. 

So basically our Swift Current physio team is awesome and loved meeting Bella. They are here when she needs them in terms of support with Bella's drinking skills and wrist function. The check in on her wrist from her Calgary doctors next step is to involve an MRI later on, since she is improving on her own with use. But if nerve damage is what is restricting her full wrist movement an MRI may be needed in the future to determine what specific type of wrist surgery is needed. For now, our Swift Current team will monitor her progress. 

Bella's academic skills are still improving as well, and this is all her own interest. No, I'm not pushing her because I am a teacher, but if you know me, you'll know I am a tad excited about her brain skills. Hehehe

She knows all her letters now and can identify them all. She knows the majority of all their sounds. She has started to love counting and asks me to count Legos with her while she builds Lego towers. She can only count to 5 on her own but can identify numbers 1-10 when she sees them written. And her animal knowledge is rediculous! She knows more than your average dog and cat these days and gets excited when she picks out a hippo, elephant or giraff in a book. Her words are so clear these days, even if she heard them for the first time...it's like she's a genius parrot. Which reminds me to try my hardest not to let an swears slip out midst a frustration moment of mine around her, because the parrot is always listening. Haha. Thankfully, my little parrot is only repeating good words. I also love that she's starting to have converstaions with me. If you understand what I mean by this, its super weird to me, but in a funny way. Her phrases and questions in conversation are getting longer and longer and I'm starting to realize mid sentence that she totally gets what I am fully saying, even if I ramble on thinking she doesn't get it...she does. She's so smart it's starting to scare me. Keeping me on my toes. 

So How Am I?

Well, my smile hides a lot. I'm open and I am honest, yes, but it still hides A LOT. I'm struggling with a lot right now and you might wonder how is it possible that I can talk about it on this blog. Well, I am an extrovert, I'm an open book. Being open about a lot of my struggles helps me deal. I beleive if you are silent and leave people guessing, they often guess wrong. So I rather be open about what I struggle with or what I need. But trust me, it wasn't easy getting to be this way however I know it's better for me. I don't want to be a person that complains and whines, I want to be strong and show everyone, my daughter, I am strong. But I'm not stupid...when you need help, you need help... So ask for it. Do what you can for your self, but when you need to, ask for help. 

I'm struggling with being told "No."

 No Kyla you can't go away for a weekend. No you can not take your daughter swimming. No, you can not plan a weekend away. No you can not take Bella to that birthday party. No you can not spur of the moment take a trip. No. 

No, you do not get a break and no you don't get a break when you want or how you want either. No you can't even plan to leave Bella with the grandparents for the weekend or a babysitter that your friend uses. They don't know Gtube care.

This is something that has been wearing me down and I am trying to relax, figure it out and try again.

I don't get a break. 'Break' is a pretty generic term, but when it means your sanity, adventure, your fun...you long for a break. No Kyla, you don't get a break. But why? Well... No one but your husband and you know how to feed Bella through her Gtube and he has to work. No one else can give her medications safely but your husband, and he has to work. No one else has been to the hundreds of physio, Gtube, venting, clinic and doctor visits but you, to know what to do incase of an emergency...and yes your hubby has to work. So no Kyla, it is not safe for you to take a break from Bella for more than a few hours, while she sleeps, or doesn't need fluids, and if she's not sick....unless your husband (that's right) isn't working. 

I'm working on making instruction sheets for everything to do with Bella's feeds, meds, Gtube venting, her needs in general for other family members. But it takes so much time, and practice and training. From a teachers point of view it's like why go through the hours of work to plan for a sub when you can just do it yourself m, even though you are practically on your death bed with the flu. It's just easier to suck it up and do it yourself.  

So I'm struggling with needing a long over due break. I can complain until I an blue in the face, but that doesn't do me any good. But how, how do I ever get a break? It's just not possible at the moment and it's starting to wear on me. The planning and prep it takes for me to just be away from Bella for even a few hours is rediculous. But that's how it goes when only me and my husband (who has to work) are the only two people who know how to care for Bella in every way she needs. And when you see the fear in others eyes while you try to explain something as simple as a gravity feed...it's not to them, it's like asking them to instantly preform brain surgery...not easy...it's scary. 

Part of me wants to give up and suck it up and just know that this is the unique cards we have been dealt. Deal with it. No, you haven't been on a vacation in 3 years and you probably won't get one for another 3 years. No you don't get to go away for the weekend with your husband just for fun. No you can't just go visit that friend you haven't seen in years because you're the only one capable of Bella's current care.

So, I'm not doing ok with all this and I need help. I need a break. As hard as my hubby, mom, family are working on this, it's difficult but hopefully we will get there soon. 

It's hard because I come from a background of 'get up and go!' Go explore, discover, have fun. My dad gave me the most amazing opportunities during my childhood to teens to 20's, to see the world, travel. I'm so happy he showed me that way of life. And now I have lived through 2 years of jail, torture and the hell of isolation with my daughter. Not only do I want a break for myself, but after being held back in so many torturous, agonizing ways...I want that break for Bella to come as well. She more than deserves to see this world, travel and explore when she wants and not be told, "No...you have to wait."

Praying those days come soon. 

This is getting to be quite the novel tonight! Goodness me. Well, go get some water, wine, coffee...this post marathon ain't over. 

Let's Talk Genetics!

We received good news from our Geneticist when we were in Calgary last! The re-testing proved what they were certain was going on, the second gene mutation wasn't found through the first testing...and this time around, it was! 

Ok Kyla, wth does that mean? 

Side note: I want to encourage everyone to ask me any questions that want to on Bella's Facebook page. Bella has such a long, confusing medical history that I know many questions need to be re-asked and many things need to be re-addressed for clarity. So ask away, I am in a place now where I can welcome any questions and not feel burdened by them. 

So let's see if I can explain the good news easily. We needed them to find this second gene mutation from Bella's "old blood" (pre-BMT blood) in order for us to be able to have a second child, through PGD-IVF (Pre Genetic Diagnosis, Invetro Fertilization). That's it. Basically the good news is, it is possible now for us to have a second child given the testing that can make sure our next child is not born with the life threatening illness of SCID. 

So in detail they highly suspected that Bella has CHH (Cartilage Hair Hypoplasia). When you have CHH, it is detected only through 2 receptive genes. So one seen from mom and one from dad. They found mine, but couldn't find Lyles. Given that the CHH specialist in Califonia reviewed all Bella's medical files, he also agreed it is very possible she has CHH so the second gene mutation must be there they just didn't find it in the first round of tests. CHH is the connection to the gene mutations causing Bella to have SCID. If they have these gene mutations they can detect them in our future embryos and make sure not to select the affected. 

Hopefully you are still following me here. 

The first set of genetic tests were sent to a lab not familiar with finding these gene mutations. And when they only found mine at first, they even suspected a new phenomenon of perhaps both mutations coming from me, causing all of Bella's rare genetic issues: CHH, Hirschprungs and SCID) 

For a second can you imagine the absolute guilt I felt possibly being the sole cause of ALL of Bella's pain and life in hospital. ALL. The 2 gene mutations (one from mom and one from dad) that caused what Bella has are, in all other cases, easy to find. So when they couldn't find the second one (Lyle's) they contemplated a spontaneous gene mutation, one they have never seen, being solely from me. 

Not that I want this to come out negative, but if your child's extremely rare genetic issues solely stem from you and you alone...that's a pretty hard pill to swallow. 

So I was thinking that was it for a while...until March 1st. 

Our Calgary geneticist got approval from our Sask. Geneticist to re-test, basically search again for this gene that they highly suspected should be there, from Lyle...in a different lab. A lab more familiar with finding these 2 gene mutations. And they did! This was such good news because it means we have a chance at successful PGD-IVF. And yes, the lab that missed it through the first set of tests is being notified of their mistake. Also, thanks to our Sask. Genetics Doctor Dr. Lemiere for approving the re-test. It cost them money to allow this, and they did, knowing the huge importance to our situation. 

The next steps

After finding the second gene mutation, they need to confirm it to identified it and use it as a marker for when we go through PGD-IVF. So they took my blood and Lyles blood again to confirm its marker. I guess you could say we shouldn't celebrate yet, as this confirmation test with take another month... but our Calgary geneticist is quite confident we can. 

Meaningful Days Ahead

17, 20, 23, 25

No, those are not this weeks Powerball...

Starting tomorrow, St. Patty's day, those numbers mean what it took for Bella to have a chance at living, surviving. 

March 17th 2015 was the day Bella started Chemo. The Chemo conditioning was needed prior to transplant to ensure her new cells could come into a clean slate and set up shop. 

March 20th 2015 was when Bella went through Sepsis because the Broviak line she had at that time was infected and showered her heart with bacteria after 4 days of chemo wiped out everything in her tiny body. 

This coming Sunday is going to be a difficult day for me. 

March 23rd 2015 was the day Bella's Bone Marrow donor had the procedure to draw the marrow from her bones to save Bella's life. 

March 25th 2015 was her Day Zero. When Bella received those life saving bone marrow cells from a life saving stranger. 

So March 25th, 2016 is Bella's Re-Birthday. Her New Life Day, when we celebrate her successful transplant one year post and her new blood that is now giving her new life and a chance to live. 

Check in on Bella's Facbook page (Isabella's Mustard Seed) for many updates and plans for her Re-Birthday coming up on Good Friday. 

And thank you again, from the absolute bottom, trenches and depths of my heart for sticking with us, praying for us, following Bella's extensive journey and truly understanding what we have been through...

Thank You. 

Sunshine & Rainbows - Day +327

Some say, "It's not always sunshine and rainbows." Welp... In this post it is! :)

We have lots of good news to share and we're super stoked about it. :) 

Those were daddy's glasses Bella's wearing in the cover photo. 

February 15th, 2016

2years 1week in Hospital from Birth

Day +327 Post BMT

Day 65 at Home

Calgary Clinic Visit•Feb. 2nd, 2016

We had a LOT go on during this visit for Bells's second check up with her BMT doctors. It started with Bella getting sick juuuust as we pulled into Calgary that Monday evening. What I first thought was maybe her being car sick turned out to be Bella showing us she could fight and get over a random (non-serious) virus/bug on her own! If you follow Bella's Facebook Page you'd remember my updates posted about that. But in short we were happy to realize that she is capable of that now, fighting viruses. The blood tests from her lines came back  negative and her nasal swab also came back negative. When the doctors run those tests at the first sight of a fever, they check for all the major big bugs to rule out anything serious. Bella took about 5 days of rest, Tylonol and extra fluids (via her Gtube) to get over what ever bug she had. 

Front Page of the Calgary Herald

Before this visit our Immunologist doctor had asked if she could recommend us to be a family who tells their story for the Alberta Childrens Hospital (ACH) Radiothon. So a senior communications director of ACH contacted us to prep for being interviewed at the Radiothon. The Calgary Herald also found out about our story and wanted an interview for their paper as well. I didn't realize we were Front Page material!  

Bella and I were in clinic awaiting her IVIG and RSV infusions when the reporter came to interview us, take pictures and hear our story. They masked, gowned and gloved to keep Bella safe. About ten minutes into our interview, Bella's Immunologist Doctor came in to update us on Bella's results from her recent Immune Panel bloodwork. It was exciting to share the good news with the reporter's team in the room with us both of us, hearing it all for the first time. 

Bella's doctor had told us that Bella's new immune system, donor cells, etc are doing so well that we can begin to lessen her isolation protocols! For the first time in Bella's life we were given the go ahead to allow her to play with friends! When the Calgary Herald Reporter heard this for the first time, along with me, and realized what that all meant, she was just in shock. And from there, made it the focus of her article. We were exstatic! 

It's such a weight to be lifted off my shoulders when I hear news like this from Bella's doctors. The freedom to rid myself of that worry is such an indescribable feeling. It's like another link from the stress chain, I can break off. Bella's doctors went on to tell us that play dates and mom groups are ok now, as long as the other children are not sick (of course) while Bella plays with them. They even said we can venture out to restaurants for family dinners, but we still must avoid highly populated areas like busy malls, waiting rooms, or crowds. And still no chicken pox parties. Haha

Since we have been home with this good news, however, we have yet to give Bella a play date with friends. But we are eager to start soon. We wanted to give her more time to make sure she was over that fever bug, and then we have been waiting for her friends in Swift Current to be healthy as well. All her future friends are daycare age, so it's a tad tricky to find a healthy, germ free friend in the middle of February. But soon!

Radiothon!

On February 3rd at 8am we were able to share our story in support of ACH and all they have done for us after living in hospital there with Bella for 18 months! 

So if I wasn't a tad nervous already, I then had to sit down beside Paul Brandt! He and 3 other radio hosts from Country 105 had a few notes about Bella and asked us questions about what we have been through and how ACH supported us, so intern, please support this amazing hospital. As much as I wanted to advocated for SCID screening to be implemented in all our provinces, the Radiothon wasn't the place for that. Also, the Calgary Herald already helped us with that angle. For the Radiothon we wanted to express our gratitude to the amazing, life saving Bone Marrow Transplant team, doctors, equipment, etc. That part of ACH is what saved Bella's life. 

The interview was quick and of course I cried, cause I'm a sap. Lyle even tried coaching me a bit prior, but as happy as I am going in, it usually only takes one word to trigger my waterfall of tears...everytime. This time I think it was the tears in the female radio host's eyes that I caught a glimps of, that triggered mine. But it's ok, the stories that ACH families share are priceless and it is so needed. The donors that give so much to this hospital need to hear what their support truly means, and that will always involve happy tears. 

I knew I was either going to ramble on and rely on Lyle to save me or cry, haha, so I write down a few notes to make sure I got out what I really needed to say to everyone listening that morning. Here are what I call "Bella's Stats." 

When I showed it to the radio hosts their eyes widened with shock, as they saw the list of 30 some services Bella required at ACH.

I also wrote down what I did not want to forget to say. Knowing so many Calgarians, Albertans were listening in during that prime time, 8am commute to work, I wanted to make sure I didn't forget some imortant words: 

"I know if I can get Bella's story out there  more people will understand how much ACH needs support, how much this hospital has kept my daughter alive, cured her and made it possible for us to bring her home.  I also want to say, for those who just can't give and it pains them to hear our stories and not be able to give to ACH, please know your effort to just understand what us medical parents and children have gone through, is priceless. It means a lot, Thank you."

I didn't get the chance to listen to our bit on air (they'll send me the link to our segment soon) but I sure did get a chance to listen to all the other families stories during the 3 days of Radiothon, and wow... 

There were so many stories that were so immensly powerful. I honestly couldn't stop crying. Happy tears and broken heart tears. But this is not just ACH pump'n its tires, these stories are powerful because they educate people in huge ways. I learned an amazing amount about so many different diagnosis, procedures, family trauma, that I never knew about. It gave me a priceless connection knowing that so much of the pain Bella , Lyle and I endured was understood by so many already, in so many ways. And in a way, that helps me a lot. And then also hearing all the success stories similar to ours just gave me this boost of happy energy knowing so many more lives of children are better because of this hospital. 

Hopefully I'll receive the links to our Radiothon interview and the Global Calgary TV interview we did aswell. Yes, that's right, we did 3 interviews in one day! Follow Bella's Facebook page for updates. :) 

Facebook page link:

https://m.facebook.com/Isabellas-Mustard-Seed-848573408556136/ 

MAC Bug Update

The Non-Tuberculosis MAC bug that Bella was diagnosed with back in September is being treated by having her on 3 different antibiotics. We found out from Bella'a Infectious Disease Doctor that these 3 medications are to be 100% covered by a TB company that supports patients linked to this disease. 

The MAC bug discovered in Bella's lymphnode back when they did surgery to test her lymphnodes for cancer, is a bug that you or I (with working immune systems) may actually have. But it doesn't affect us, cause our immune systems work. But when discovered in someone with no immune system or immune compromised, can be deadly. Bella was to be on these antibiotics for at least one year, once her new immune system is competent. So over a year on these meds...very expensive. To hear that they are to be 100% covered was wonderful news!   Currently our pharmacists in Alberta and Saskatchewan are looking in to getting this all figured out for us and hopefully we'll receive back pay for what we have already paid. There is a chance that because we are in Sask. The one med may not be covered, but at least 2 could be. And I did confirm it was quite a miracle that the doctors discovered this MAC bug in Bella when they did, because untreated, it could of killed her. They found it by happenstance when they took the lymphnodes out to check for PTLD post Transplant. The fact that they also found this MAC bug at that time, saved her life. 

I've discovered a lot about the coverage our province provides since we have been home, and it's not as good as Alberta. There are big differences, cost wise. So those medications I just talked about, all 3 are covered in Alberta. But in Sask. Maybe two are, and our pharmacist is jumping through hoops to figure it out. Also Bella's highly specialized formula, formula you can only buy from a pharmacy, not covered at all in Sask. Bella's dietician tried applying multiple times stating Bella's is still 100% reliant on formula via her GTube. But Sask. Denied our requests. Alberta Homecare coveres 100% of Pedialyte cost...not in Sask. And Bella requires at least 300mL of Pedialyte per day to help with her fluid levels and balancing her electorates. Pedialyte is a prescription for Bella because she can not properly balance her fluids given a few reasons. 1. She has no colon. 2. Kidneys recovering from BMT. And 3. All the current medications she is on. Without Pedialyte her potassium levels suffer and her sodium levels jump around. These can not be let into dangerous levels, they must be maintained with no major drops. 

So I guess that's one little rain cloud amoungst our sunshine and rainbows. Ugh. But we'll do what we can for now.

Back to good news...

Bella is having surgery to get her Broviak Central Line taken out! This will be Bella's 10th surgery, but it is a very good thing. She has had this line (that they use for all blood work) since March 2015. It has served her well, but she is so much better without it. 

This is an older photo of Bella's central line back when it went through a repair.

So what does it mean to get this little sucker out?! 

It means I don't have to tape half my daughter's body with Saranwrap and waterproof tape for her baths! It means no more stress and worry about horrible, deadly infection stemming from the line like the one that almost killed her and sent her into 4 sepsis attacks last March. It means I can take her swimming and actually go to mom and tot swimming lessons with her! It means no more paying for all the expensive supplies that go along with having a CVC line sewn in your chest! Very good news. 

Taking this line out also means that Bella will have to go back to getting poked for blood and infusions. However, after much discussion with Bella's doctors, the risk of leaving this line in her chest is way way too high for infection and so even though Bella has bloodwork more than once a month right now... She is progressing and is way better off with a few more pokes than risking leaving this line in. 

I'm sure I will bite my tongue on that the second they have to poke a needle in her arm, but I know and her doctors know she is progressing towards less and less bloodwork, which in turn will mean less and less pokes. 

I've also received some very helpful tips from other parents who's kids currently need to be poked for blood work and I'm sure it will all be better than leaving that CVC in. I plan on making a pre-poke package to help Bella, containing everything that could possibly help her through getting a poke for bloodwork. Please send me more ideas if you have some! So far I know it will help to make sure she is quite hydrated prior. I remember the blue band they tie on hurts  her, so I'll remember to dress her in a loose long sleeve shirt so the band does not pinch her skin. We can ask the nurses for numbing cream prior to the poke on the poke site. And of course a reward for after, to help dry those tears. So far I think stickers or her current favourite treat...French Fries will hopefully help. 

I do know, I may be going back into that hard shell of mine who finds it extremely difficult to hear about your healthy child getting their once a year flu shot and how horrific it is for them. 

Between the 3 Broviaks (surgery to place and remove this type of CVC) and 2 attempts at PICC lines in Bella, that all took place in Sasktoon RUH within the first 7 months of Bella's life...

She endured hundreds of pokes. Hundreds. It was horrible and too painful to go on and tell you where I was going with that information. I'm just going to leave it. 

Genetics

More Good News!

We are very very excited to finally meet with our Genetics doctor come March 1st. She emailed us last week stating the second round of testing they did with Bella's old blood came back with a result!  This means they have found a promising second genetic mutation that could mean confirmation of a diagnosis with Bella and in turn allow us to try for having a second child through PGD and IVF. No... Not part of the next X-Men movie.  We are very excited to get the details from our Gene doc when we meet with her in person. Stay tuned. :)

Bella is doing quite wonderful this month and we are so proud of her. She is walking more and more and more! Lyle actually made the call to hide her push cart and see how she does. Now Bella is walking everywhere, no pushcart!  She walks all around the house without falling now and is not requesting her push cart. It's so beautiful to see her strength in this way. Even with all her energy, her naps have been getting shorter. She's only napping for about an hour now where she used to nap for a solid 2 hours at least. But she is also sleeping so well through the entire night, we are quite spoiled that way. Currently her favourite activities right now are colouring and anything to do with letters and ABC's. So much so that this little genius knows 17 letters in the alphabet right now! And she's only 2 years old. Ya, I know! It's like, should she be on the Ellen show for this, or what?! Ha ha. 

My curiosity started when she began playing with her foam letters and calling them by name as she held them in the air. At first it was, "Momma, a B!"

And I thought oh, ok she knows one. Maybe she saw it on Super Readers cartoon today. Then, "Momma, an R!"

Yep, she's holding up an R...hmmmm, is this coincidence? 

This happened more and more. And so of course the teacher in me got very excited and I needed to test her knowledge of all the letters. We started singing ABC's and looking at all the letters around her play table as we sang. She would want help drawing the letters on her Elmo ABC app on the iPad. That was huge cause all she ever did on that iPad was Toopy and Binoo cartoons. Now, straight to Elmo's ABCs writing. And now, she knows 17 letters, all on her own! The letters she knows are: a,b,c,d,f,i,m,n,o,p,q,r,s,t,u,w and x) 

Thank You Unit 1 Staff at ACH

During Bella's February clinic visit we finally were able to drop off our Thank You to Unit 1. (The Unit where Bella had her BMT). I was very happy to finally find a way to express our thanks to all the nurses, doctors, specialists and staff that made this unit Bella's home for her. The staff there made the unit for Bella and I, a happy place where you would expect so many tears, they made me laugh amoungst heart breaking stress, they made it possible for Lyle and I to bring our baby girl home. How do you even begin to think any 'Thank You' will be good enough. I hope what I made for them shows them our endless thanks for all they have done. 

I created baskets with over 100 personal Thank You cards so that every staff on the Unit could read or take a card. Each card had a unique momment that we wanted to give thanks for. Some moments/memories are from me, Lyle, Bella and our parents. Many of the moments are something a nurse, doctor or unit one staff did or said. 

Although so many moments have a specific Unit 1 person, nurse or staff connected to it, I made them generic so that even if it wasn't that specific nurse that was a part of that memory, the nurse that does read it knows what her and her colleague have done for us and probably other families as well, that means so very much. 

Here are just a few more examples of what I wrote on the cards:

Rare Disease Day is coming up! This will be February 29th, 2016. This is a significant day for Bella because she has a large amount of rare diagnosis: SCID, Hirschprungs Disease CHH and NonTB-MAC. This day is very important and I hope to help more people become aware of what this days means to people like me and my family. If you can support Rare Disease Day, then it means you support families and all they go through with the diagnosis of rare disease or what can be worse...no diagnosis. For Bella, this support helps keep the Genetic programs in place. We have relied so very much on our Geneticists to understand what is going on with Bella so she can be treated, so we can support her growth and hopefully avoid SCID with our next child.  

Please try to find out what your community is doing for Rare Disease Day, and if the only way you can show support is by wearing jeans, that's great too. Wear jeans for Bella! There are many sites that can help you learn more about Rare Disease Day: Global Genes, the Rare Disease Foundation, and Rare DiseaseDay.org. 

Telemiracle 40! 

https://www.telemiracle.com/index.cfm

Telemiracle is a HUGE Saskatchewan charity that supports Saskatchewan families needing medical assistance or equipment. The Kinsmen have supported my family in big big ways for Bella and all her medical requirements and we can't wait to give back to them and show our appreciation.  That's right folks, we are doing another interview! But we are happy that this one is in support of our own province, Saskatchewan. CTV will be coming to our home before Telemiracle airs, to record our video testimonial. We of course can not travel to Regina and be a part of the big stage show, as much as we'd love to do that, it would be too risky to have Bella amongst that large crowd of people. So Telemiracle's filming crew from CTV are coming to us! Stay tuned for this awesome video of me crying again...just kidding. We can not wait to help support Kinsmen and share our story so that more and more people understand what this amazing foundation has done for our province and families like mine and our Bella. 

Please support Telemiracle 40 in Regina, March 5th and 6th this year. And stay tuned for our Video Testimonial. :) 

Learning to Walk Again, I Believe I've Waited Long Enough • Day +306

If you can listen to Walk by the Foo Fighters right now, do. It's so fitting for Bella's life after coming home, it's like her fight song. 

January 25th, 2016

Day 44 at Home

Day +306 Post BMT

Let's start off with some great news...Bella is loving toast! For the past week she has really gotten a lot better with her eating and drinking again, since the setbacks from coming off of the steroid Predisone. So her favorite food right now is toast, she'll eat at least 3 slices a day, one at every meal. And it's adorable how she says toast, like "tewsssst." 

A quick back-track to Bella's first Calgary clinic visit since being home, that was on Jan. 5th. That appointment went very smooth and once again Bella was an excellent traveler for the long drive. Our main concern at that appointment was Bella's extremely distended abdomen, vomitting issues and what it all meant? We were reassured by Bella's doctors that it was all an effect of coming off the strong steroid that she was on for so long. They had seen it lots before and that it could take her a while to get back to her 'normal.'  As reassuring as it was to know the cause, it made for a hellish month at home. Bella's stomach was so distended it scared us. We couldn't sleep and it was like walking on glass with every symptom. I think I called her doctors quite a bit so they could help me through and ease my worry. But to worry, every other second, "What if we need to go to Emergency? What if her bowel is perforated? What if it's nothing but what the doctor says it is? What if it's actually a blockage? When will it stop?"  To live with all that worry, flooding back once we got home, was torture. Thankfully after a solid month it finally went away. Unfortunetly it caused a lot of setbacks that were extremly hard on Bella, Lyle and I. Bella had to be put on more medications. Her eating and drinking were affected and we had to digress all the way back to continuous gtube feeds at night, and because of the belly distension issues, it became difficult for Bella to keep up with her walking skills. 

The worst of it all, her Gtube site. The extreme distension caused relentless granulation tissue around Bella's Gtube site and the only way to fix that...burn it off with Nitrate. Basically hell for any parent that has to do that to their own child. Bella had this happen twice before while in hospital but the Gtube nurse treated her for me, and she was quick about it, very skilled. Now that was all on me. Multiple times I had to hold my daughter down, and burn her tissue off.

So hold burning Nitrate to her tissue. I'll give you a minute to swallow what that pain might feel like.  Hell.

Lyle and I were puddles of tears everytime, but if you don't treat granulation tissue it gets worse and it is also painful if left and could get to a point of needing surgical removal if left. 

After 4 treatments every 3 days it finally started to look better and now that Bella's stomach distension is gone, the site can heal better as well. 

The Nitrate sticks and supplies I need to treat Bella's granulation tissue. 

The Nitrate treatments were not only hard because I had to put Bella through pain but they also bring up memories that are hard to deal with. Many of you may know the term 'triggers.' It is difficult for someone to talk about their own triggers but if I'm strong enough to use these experiences to educate people around me, it will help me heal in the long run. 

I'll tell you this, it's going to be hard for me to talk with you, reminice with you...who ever you are to me when the conversations happen. If you are family to me, a friend, another medical parent, it's going to be hard. One simple word could throw me off, back into a hard shell that cuts you off. If you haven't been through what Bella and I have been through it's going to be hard. I understand that could possibly be every other person on this planet. That's why I'm telling you this now, I'm working on my healing and I know it's going to be difficult but someday I want to have that conversation that doesn't bring up triggers, doesn't close me off and won't break me down. I'm getting better, I can watch other children eat now. I remember when I had already spent days, weeks in isolation with Bella, never leaving her hospital room. Those days were enjoyable because I didn't have to see other children capable of feeding themselves when my daughter couldn't. It's hard to express what that pain feels like, when a trigger comes up in your life. But I think more people need to understand. It's about spreading compassion, right? Try and understand someone's past so you can help them heal, compassion. For me, try to imagine an intercom system set up in your home and place of work. Everytime a special bell rings you know it's followed by a voice telling you,"CODE BLUE." 

And after whitnessing your own child being intubated, other children being intubated, you physically see those images in your mind every time that bell rings and those words are spoken. I lived that for over a year of my life. I remember the nurses who cried outside my daughter's hospital room because they lived that too. Triggers feel like that intercome is set up in your home for the rest of your life. And I'm trying to dismantle it. 

Bella watching her dadda move the massive snowbank in our front yard. 

We finally got some snow around here!

Ok, so back to our January Calgary clinic visit. Everything went smooth, Bella received her IVIG and RSV infusions, we saw all of her doctors and specialists and since then have had a good time back home on our acreage. Bella's immunologist gave us wonderful news about her December 2015 Immune Panel. (The large monthly blood draw)  Bella's new immune system is right on track and even better than normal with some cell growth! Her immunologist couldn't be happier with how all her new cells are doing. Very good news. Now I know that is a very general statment but it is difficult to explain the details. However, in a post BMT, SCID case, that is a good problem to have. :)  

 I have created better questions for our next visit to Calgary when we review Bella's January 2016 Immune Panel with her Immunologist. Then I will have more detailed updates about just how good her new immune system is doing. But to give you an idea of how good, at our last appointment, Bells's Immunologist even utter the words, "Coming Off Isolation." !!

Ya! that's huge. So in February we will have an updated timeline for when Bella could perhaps start seeing her own cousins and playing with children her age! 

I of course still want to keep Bella protected untill she is allowed to start immunisations/vaccinations. And she of course will continue to not be allowed around anyone sick. We need to remember her immunizations don't even start until her Immune System has shown it is working completely on its own with no medications, etc. Until then I feel quite awkward when greeted by friends. I'm trying to think of ways around this kind of incident, instead of nodding silly or bowing all akward and saying something to someone that actually does sound like another language they don't understand. "I...Uh...Can't shake your hand, protecting my daughter, she has SCID,on protective isolation. Nope, uh, no hugging either, uh..., No germs please."

 Ya, awkward. So if you are a part of a converstaion where someone is like, "What's Kyla's deal? She doesn't shake hands or hug." ha ha  Please help me get the word out that no, I haven't made some random cultural greeting change, I'm more like a germafobe not wanting to risk bringing germs home to my daughter's clean environment. 

Back to Bella's improvements. She is doing wonderful at home. Besides the recent steroid set backs, she has gotten back on her feet again this week and is walking unassisted again. Although she loves using her rolling walker, more often she is now standing on her own, proclaming out loud that she is standing by saying, "Momma! Wow!" and then proceeding with a few steps. 

I feel, as exciting as it was to see her take her first steps on Decemeber 1st, I didn't give her the amount of credit she deserves for this accomplishment. Although she was 2 when she started walking, given what she went through, the amount of surgeries (9, five of which were abdominal surgeries) and the timing of those surgeries, chemo, BMT...they were ironically (given the unique circumstances that brought on each surgery and procedure) spaced apart so that just when Bella worked so damb hard to get to her next milestone (ie: sitting up, crawling, pulling herself up, walking) a surgery/procedure set her back.  Her Physio therapists and doctors have repeatedly told me they are astounded at how early she was able to walk given what she had gone through.  It is a testiment to her amazing strength at such a young age that she taught herself to stand up and walk by the age of two. Bravo baby girl, you deserve a large amount of applause for this accomplishment. 

These are a few of the notes written on the Postcards for Bella and her Bone Marrow donor (who we will be able to meet in 2017.) These particular notes are from students I used to teach and students from the school I used to teach at in Hodgeville, SK. Lets just say I could not get through all the beautiful and sincere notes without a whole box of kleenex. 

This makes me so so happy. Not only do I feel extrememly loved by these people and friends, but I know that my mission to inform more and more people about becoming a Bone Marrow Donor is growing as well. To see this type of compassion from such young students is so endearing. Thank You Hodgeville.

 Recently, since we have been home, I  finally had a chance to go through the 20 boxes of baby/child clothes we had piled in our basement. This picture and sticky note represents my HOPE, our HOPE, to in the future, have another baby. I beleive that if you write it down or talk like your hopes and dreams will happen, theres a better chance that they will. This box is full of newborn baby clothes in hopes that one day we can have another baby. 

We continue to wait on reports from Bella's Geneticist. We know that rolling the dice and risking having another baby subject to SCID, is not an option. To put another child through the pain Bella has endured is unimaginable, it won't happen. So this is the route we must take to prevent that tragedy again. We understand our hands are tied. Because of what Bella has, and has gone through...we can not just get pregnant when we want, plan to have another child when we want or build our family when we wish, we are now another couple that does not get that freedom, that choice, that beautiful luxury. 

Lyle and I are extremely thankful for the knowledge our specialists have this day and age to handle unique genetic cases like ours in hopes that pre-genetic testing through IVF could in fact support us in conceiving a healthy baby without SCID. For now, we rely fully on the genetic testing of Bella's "old" blood (her blood before her BMT), our blood and the expertise of our genetic specialists to find the genes which contributed to the cause of SCID in Bella so that we can see more clearly how Bella was born with SCID, which in part can help us conceive a second child without SCID . 

 For now, we hope and pray that the results from Bella's genetic testing come back clear and complete. 

If I haven't caught you up lately on this particular issue I appologize. Believe you me, genetics and all it encompasses is hard to wrap your head around. Trust, me, I've been trying to for the past 2 years. 

In a nut shell, Bella has a unique form of SCID, not one of the typical 20 kinds. Not a kind that they have seen before or shows up easily in their labs. On top of that, they speculate she has Cartilage Hair Hypolasia which would  and could explain her having SCID and Hirschprungs but the second gene that says she has CHH (To have CHH both parents have to be carriers and contribute one gene each) has not been found yet. Anyone that has CHH has 2 genes, easy to find genes. But in Bella's case they can't find the second one, making it harder to confirm she has CHH. And in part clouding the facts as to how she was born with SCID. 

Then, the reality of her having all 3 of these rare conditions combined, spontaneously from an extremely rare random gene mutation is also almost certainly so rare that it should never happen again, but too risky to risk not knowing the actual cause. 

I know, that's not exactly "in a nutshell." 

Let me try again...

If genetics does not find what they need to find from Bella's old blood testing (the results we are currently waiting on) then we are basically told we can not have any more children unless we are willing to risk them having SCID. 

Bella's genetic stats are breath taking. The combination of her rare conditions are so rare that her specialists are very confident no one else in this world has what she has. Which leads them to saying theres no way it could happen again. The rarety of the spontaneous gene mutation that caused Bella to have the combination SCID, CHH and Hirschprungs is such a genetically mind bogoling occurance that they actually can say, there should be no way this could happen again with a second pregnancy. But when you can't clearly find the genetic make up that caused Bella to have these rare conditions, you can't see it to prevent it either. 

In softer words, my heart is breaking. It breaks with every other family out there not able to have kids at all. I feel that pain, and then get slapped back to the realization I have one child already, I'm already blessed with one. So, shut up Kyla... right? Right. But thats not totally fair. I know a lot of medical mommas out there who understand. Is it any more fair to be angry and hurt by someone who has a child when you can't, and forget the torture, pain and extremely unique hardship they have had to go through to keep their only child alive? 

I want a chance to have a baby without being dropped into a medical hell of continuous torturous agony. I think we deserve to experiemce something lighter than that. 

In all reality I guess I'm talking about this a tad too soon but it'll come up any time genetics comes up. And of course Lyle and I long to have more kids, a second chance that can help heal our pain from seeing what no newborn should ever have to go through. And instead feel the healing love a second baby could bring to all three of us. 

I'm going to end this post here knowing that I'll have more to add in just one weeks time! We will be off to Bella's second Calgary appointment since being home in Sask. 

        More good news will come!