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.
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:
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:
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:
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.
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.
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.
