Nadia's Quest

We were so scared.

An urgent trip to BC children’s hospital in August set the scene. A weeks worth of tests without a diagnosis was scary. We were sent back to Kelowna with a few potential diagnoses to choose from. We learned as much as we could about all of them, decided which one was scariest, and hoped that wasn’t it. When we returned for follow-up appointments Nadia was given a diagnosis of Dyskeratosis Congenita, something not mentioned before, and ends up being even scarier than the other choices.

What does it all mean? Will Nadia survive?

Well, no, not without a bone marrow transplant. And this won’t cure her, but will prolong her life, if she survives the transplant.

Pardon?

A ‘typical’ bone marrow transplant can’t be done. Her DC makes her too sensitive to the chemotherapy and total body irradiation. But we do have other, lower dose options.

OK, great!

But, they are risky.

Pardon?

Well, only a handful of transplants have been done using the candidate protocols. We have to choose one of them, and make some modifications we think will help.

So my child is a science experiment, but not in a fun way?

yes.

What are her chances?

We don’t know. And it depends if we can find a suitable stem cell match from bone marrow and cord blood registries.

How hard is that?

Hard. Especially for a child with mixed ethnicity. And even if the transplant is successful, there is a risk of acute and long term side affects. This is called Graft Versus Host Disease.

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Above is the rough dialog of what we lived with from October to February. It was at times debilitating fearing what was to come.

We definitely went in fearing the worst, and scared of an unhappy outcome.

At about day -9 Nadia was hooked up all day long to IVy getting her chemo drugs, pumped with steroids, and stuck in her room (pre Isolation Room days). The whole day was essentially a meltdown/tantrum for Nadia. She behaved in ways she never had before.

On this day I broke down. I could not speak. I could not stop crying. I imagined our future with Nadia was going to be filled with more days like this, followed by Nadia sick in bed, and potentially sick the rest of her life, if she survived at all. I thought Nadia’s day-long freak-out behaviour was the best of the time we had left.

It crushed me.

I’ve never been through a lower low.

Tara had to look after us both. (Thank you Tara for your strength!!!)

This experience was not documented before in the blog. We made great efforts to keep the content uplifting if possible. We were simply not expecting what came next (in a good way!).

Nadia quickly became accustomed to the small space. To the amazement of everyone, Nadia did not become sick like everyone expected, and she flourished. She was active until she fell asleep at night. She ate like a champ, and she grew almost 2 inches.

AND, she engrafed. She had a bone marrow stem cell transplant using a technique in its infancy for a rare disease that is still not well understood.

We are so fortunate.

We are blessed.

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And we are grateful.

Thank you to all our friends, family, neighbors, doctors, cleaning staff, care givers, nurses, kind strangers. Thank you everyone for helping us get through this.

We live in a community. And not just the obvious definition of community, being people we live near. But ‘community’ in the most beautiful sense: the feeling of fellowship with others, as a result of sharing common attitudes, interests, and goals — saving Nadia. Through our experience living Nadia’s Quest we have grown to appreciate community in a whole new way. We reached out for help and the community delivered. People from Kelowna, Vancouver, Wynyard, Elfros, Mozart, Foam Lake, BC, AB, Canada, and around the world helped save Nadia’s life. From people locally watching our house; to the people who provided day-to-day care for Nadia during transplant; to the people from our workplaces who provided support; to people letting us call them at any hour to talk; to people who read this blog, follow twitter and facebook; to people who sent care packages, messages of encouragement, cards, calls, emails, sharing our posts with your networks to help raise awareness about bone marrow registries and blood donations; to old friends reconnected; to the many blood donors who gave up their time and blood so Nadia could have 10 transfusions; to those who started donating blood because of Nadia’s quest and swabbed for bone marrow registries and convinced others to do so; to the international community of DC patients, families and physicians; and to the mother/child team from somewhere in the world who donated their cord blood to give Nadia a second chance at life – to our community, who we now consider neighbors and friends, Thank You.

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Nadia having a smoothie as we drive home from Vancouver.

Nadia is not sure about these bunnies!

Nadia acting like a normal, awesome 2 1/2 yr old 🙂

Well it has been a while since we updated! a LONG while and i apologize. i remember back in October when I first started following a few blogs of people who were going through BMTs and hadn’t updated their blogs in a long while and I would get so mad. But I get it now why they didn’t, there is nothing to write about. Which you as a reader have to understand is such a good thing.  When we were first admitted to hospital back in February to start Nadia’s chemo, we met with the dietitian.  She said to us in the meeting, ‘you want boring, you want your life to be as boring as possible for the next year’ at first I didn’t really get it, I want Nadia to be normal, not boring!  I get it now, boring is normal.  It means life is just chugging along and there are no hick ups and set backs.  Up to now, other than our painfully slow start, Nadia hasn’t had any hiccups.  We dodged the Noro virus that decided to make an appearance for the first time in 15 yrs on the oncology ward at BCCH and so far (touch wood) have dodged other things like colds and flus.  This is pretty much unheard of in a BMT patient’s life.  Most patients will come down with some sort of virus or infection by this stage of the post BMT journey.  Don’t get me wrong this could still happen, Nadia’s immune system is super fragile, if she does get something like a cold or a tummy bug it will take her out – she will most likely be sick longer than the average person and more than likely be hospitalized to be put on IV antibiotics to giver her a boost.  Her entire immune system will ALL go to fight off whatever is attacking her leaving a wide open door for other viruses to come in and and start reeking havoc on her. Unlike an immune system of a ‘normal’ person where the immune system sends in a special forces team to kick the crap out of a particular virus or bug leaving the rest of your immune system to stand guard to fend off other bugs that try to infiltrate.  Make sense?  This is why we have been living a boring life. Limiting visitors, OCD hand washing, and living in a bubble.  Her system needs to learn how to fight off bugs and germs.  So, boring is what are life is at the moment.  I know we can’t live like this forever and she will get sick, but the longer she is in her bubble the stronger her immune system will get so that it will be better able to fight off germs. It is also constantly on my mind of every minute of every day that a fever could spike.  There is no way to shed this feeling, it hangs over you and you are constantly on high alert.  It is exhausting, because you feel like if you let your guard down it will open up the highway of germs to your kid.

So our life right now is BORING. We get up, eat breakfast, watch some TV while mommy enjoys her morning coffee, play outside for a bit, eat lunch, nap, wake up, daddy comes home, play some more, eat supper, bath, play, bed time, repeat.  This is what our day consists of, so really there has been nothing to write about.  I could fill you in on her daily 2 yr old tantrums, but that would involve me reliving the moment where mommy almost looses her $hit. And nobody wants that. 😛

Perhaps I will write more when we start potty training it may be the thing that breaks me, god knows the temper tantrums have me on thin ice.  HA!

Last Friday we got her blood work done and to say I didn’t cry like a baby would be lying.  I had to ask our hematologist nurse to look at her neutriphil (anc) number again because I thought I was reading it wrong.

so here they all are in black and white for the ENTIRE Internet to see and read:
WBC  4.6  (normal range 5.6-15)
HgB  128  (normal range 105-135)
Platelets 277  (normal range 200-490)
ANC (neutriphils) 1.84 (normal range 1.5-8.5

If this was her report card it would read EXCEEDS EXPECTATIONS!! well I am pretty sure that is the grade her hematologist would give her and if not, I would be right in there arguing for a higher mark!  😛

as you can see her WBC is still shy of the normal range, but I’ll take it.  Remember when it finally started to register and then just hung out there at 0.2 FOREVER?!?!?! It was like time stood still for 52 days. It is definitely hard thinking back to those days.

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Nadia enjoying her (thoroughly pre-screened for germs 😉 )

Nadia goes down a big slide without any help!

Today is 100 days since Nadia’s stem cell transplant! Day 100 is a milestone in the BMT world. We continue to ramp down her medications and hope for the best. Why not celebrate with us?

Thanks for joining us on Nadia’s Quest. 🙂

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Camera Roll – Nadia has her cake and eats it too 😉

Nom Nom Nom

The Metro Vancouver published an article today on Nadia following up on their previous story. Much to our surprise Nadia made the front page! Our cover girl. 😉

Thank you Sam for sharing our story and helping raise awareness for stem cell and blood donations.

We had our last appointment at the outpatient clinic last Tuesday the 20th, where they gave us the green light to return back to Kelowna.  Let’s just say we ran outta BCCH as soon as they gave us the go and didn’t look back, and maybe even had selective hearing as we power walked down the hallway. 😛 Team BMT continue to be happy with her counts and her sunny disposition. We have a scheduled appointment for Nadia down at the coast at the end of July. In the meantime we will go for blood work here in Kelowna next week to see where she is at and asses after that if they want us to go weekly, bi-weekly or monthly for bloods. Fingers crossed Nadia’s counts will continue to go up where they need to.

We came home to a lovely welcome home poster plastered to the garage door made by Nadia’s daycare. It was such a nice welcome home surprise. We are loving being back at home.  Nadia was like a kid in a candy store when we walked into the house. She was running around, looking at things, touching things – she was thrilled.  She is able to reach things on the counter that she wasn’t able to reach before.  According to our very accurate home made growth chart on the wall, she has grown 2 inches since January. I knew she had grown, but that much? I was shocked. We are feeling very blessed that she has grown and put on weight since January.  We had no idea how Nadia would do physically during her BMT. We expected her to be tired, lethargic and loose all of her hair.  She was none of these things, in fact she was the opposite and we are so happy and grateful as we know this is not the case for every BMT patient out there. Nadia thus far has been the exception to the rule this far in her little quest.

Being at home has been great so far.  We are all enjoying being together, in our house and running around the backyard.  Nadia is still not allowed to go into crowds or busy public places, if we do go out to someplace public she wears her mask. But to be truthful we haven’t really been out much, I am sure you can empathize that you would rather keep your immune compromised kid in the confines of your own little bubble than expose them to the dirty public. Plus we all know bringing a 2.5 yr old out to public places doesn’t always end well, so it is just easier to avoid it. In some ways I feel it is better that she is so young and not knowing she is missing out on hanging out with her friends and having play dates. On the other hand she is missing out on interacting with kids at daycare and putting her in things like music and swimming lessons. I just keep telling myself she has the rest of her life to be dragged to music lessons kicking a screaming. HA!  Having said that we do go to the park as much as we can, and we are limited the time of day we can go. The chemo she had before transplant and some of the drugs she is on makes her more sensitive to sunlight, not to mention the disease itself, and because of the BMT she is also sensitive. And when the park starts to get busy or I see some kid with a runny nose, we are outta there!

Last week we got an email from her doctor letting us know that her 2nd chimerism test came back as 98% donor. Remember the first one said it was 91%. What does this mean? It means that only 2% of Nadia’s old bone marrow is left in there and the rest is all the new stuff doing dance dance revolution. Not sure what that 2% is doing, but what I picture is it just watching the dance dance revolution going on and wondering what the H is going on and trying to find the nearest emergency exit.  I do want to point out to whomever is reading this trying to do research on BMTs, Nadia’s Chimerism test was done through periphery blood draw i.e. regular blood sample. In cancer cases they usually do another bone marrow biopsy for this test to make sure the cancer hasn’t come back or stayed around. When Nadia was first diagnosed back in September they used a bone marrow biopsy to help diagnose her and find out what the H was going on.  I can’t remember if they will need to do another one on Nadia at the 6 month mark and yearly afterwards. Something we will ask next time.

We met with Nadia’s Kelowna Pediatrician this just to touch base post transplant and bring him up to speed with her.  He was pretty happy to see how she was doing. He is such a great doctor.  He was also pleased to tell us Nadia is now 25% for height (and that was on the fat kids chart, not the premie chart).  Take that stupid genetic disease and bone marrow failure.  HA!

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Bathing without here CVC line. We ask Nadia where here noodles are and she shrugs (??)

Einstein assuming his usual position – a dog’s life in the sun.

A noodle free life. Our monkey is back!

Nadia’s version of ‘ice cream’

Seeeee Saaaaw

‘I am a robot’. Tara can hardly contain herself and this video gave people nightmares!

Today Nadia had her CVC line removed. The operation went well and without any problems. We have an appointment next week, and then home! Of the four blood levels we have been following closely, two of them are in the normal range. Nadia’s white blood count is still low, as well as her ANC, but are still on the rise.

We are now a family reunited. Our dog has been separated from us since January. Tara and Nadia are both sooo happy to see him. 🙂

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Camera roll

Tara and Einstein

Nadia and Einstein

Nadia and Einstein again

== Counts ==

The doctors are happy with the way things are progressing with Nadia. And this makes mommy and daddy ~really~ happy. 🙂

Some of her counts look like they might be starting to level off, but they might still be increasing slightly. Yesterday her WBC = 2.7, and this is on the low side, although the low end of ‘normal’ seems to be different depending on the reference you look at. For newborns it can be 9, for adults it can be 3.5, my notes has 7 as the ‘mid range’. So there is still room for growth here, but our doctors do not appear to be worried. We just have to be careful, stay away from crowds, and try to be as clean as we can for now.

Her ANC = 1.24, and this is about half-way to the 2.5 value we are hoping for. Again, this count means we stay away from crowds and screen visitors for now.

Nadia’s Hgb = 104, and this is edging closer to 120, and healthy females usually have >120.

As for platelets, Nadia is an all-star! Yesterday she had 205, which is pretty much the middle of the normal range. She is a champ!

==Tapering CSA==

In order to prevent Graft Vs Host Disease, Nadia has been taking the immunosuppression drug cyclosporin (CSA). This is also called her Anti-Rejection drug. As of this week, she is being weaned off! It will take about 10 weeks until she won’t be taking it any more. We will have to keep an eye out to see if she starts showing any signs of GVHD, and hopefully she won’t. We are told to look for an angry rash. This rash could be her new immune system attacking her skin. If this happens, her other organs could be under attack too. They also said to watch her poo … yay … :-p

Tapering off the CSA is a big step towards living a ‘normal’ life. This is exciting.

==CVC coming out==

Perhaps most exciting in the short term is that Nadia’s CVC line is scheduled to be taken out on Tuesday! The CVC line is the ‘noodles’ that emerge from her chest. This line goes all the way up her ribcage (under her skin), up to her jugular vein, where it enters the vein and goes down to her heart. This is the line that IVy was hooked up to and was used to deliver drugs and fluids. This line has also been used to take blood samples so that we could get the counts, which ended up as wonderful graphs I know you all love. 😉

We have a surgery booked for Tuesday where this line will be taken out. Apparently the blood doctors used to just yank them out. I guess someone, somewhere decided it was too cruel to do it this way, which is kind of ironic considering Nadia was given poison (chemo drugs) right into her veins and kept in solitary confinement for 50 days! How cruel was that!! 😉

Sometimes these lines come out by accident, such as getting snagged on a slide or the kid pulling it out and handing it to their father. So, we don’t anticipate this to be a major surgery.

The next step will be home (!!!)

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Meow

Weee!

Nadia’s morning meds. The two on the left are Magnesium. Her CSA (far right) causes her to be short on Mg, so she needs top-ups three times a day. The CSA on the far right is her anti-rejection drug. It is the most bitter, so we add some chocolate syrup to the syringe. She gets CSA two times a day. The middle syringe has antibiotics in it. She gets this twice a week as a precaution since her immune system is so weak still.

More Achoo

Ninja in training

Nadia learns soccer (the father-son team was so kind and accommodating for Nadia crashing their game. Thank you strangers!)

Well we are at Day +66, i cannot believe it. We had our clinic appointment last Friday, my first time taking Nadia on my own as Thor returned to work last Wednesday to ‘bring home the bacon’ and if you caught the tweet and FB updates you would have seen her blood work numbers updated and graphs accordingly.  But I will list them again for those of you who don’t remember or can’t read graphs (no judgment here)
WBC 2.4 (normal 5.3-16.0)
Hgb 102 (normal 105-135)
Plts 201 (normal 200-550)
Neutriphils 1.07 (normal 1.5-8.5)

As you can see, she is kicking some serious A$$ in the platelet department, (just to remind you when she was diagnosed in Sept 2013 she had a platelet count of 6) And her Hgb is just about in the normal range, needless to say, she is the life of the party and her mother needs a nap as much as she does, maybe more.

Clinic appointments look like this, we arrive at the hospital, check in, put our name on the board, then get ushered into a room to ourselves. Even though Nadia is wearing a mask, because her immune system is still so fragile (pronounced the Italian way to make is sound fancy) crowds of people and hospitals are not great places for Nadia to hang out in. So we get the royal treatment and ushered into a room right away.  Still waiting for the red carpet to be rolled out for our arrivals, but haven’t seen it.  Then we wait for a very nice nurse to come in and take a whole whack of blood from Nadia, ok so it’s not a whole lot but it still seems like a lot.  Then they weigh her, take her temperature, blood pressure and height. Then we wait for the lab to do its magic and count all of Nadia’s bloods. This takes about 20 mins or so. Then Team BMT usually comes in to have a chat and go over the results. Friday was a bit different, Nadia’s nurse practitioner came in a bit early to say hello, and offered to bring in some play dough toys. I was expecting a cookie cutter and a wee rolling pin, but she brought in the entire play-dough box.  Nadia of course then insisted on dumping out the entire thing onto the desk.  Lucky for us the nurse practitioner got side tracked with playing (as you do with play-dough, it’s just so much fun) then our Hematologists came in. Nadia of course asserted her ‘leadership skills’ and had them all playing with the dough. I have to say it was a pretty awesome sight to see, 3 highly educated people hanging out, playing with play-dough and a 2.5 yr old. At one stage Nadia had enough (we all continued playing with the play-dough though) and she just started running around the room, then she cracked her head on the desk (thank-god she has platelets to spare), rubbed it, let out a little cry and put her arms up to one of the hematologists for a snuggle.  He obliged, and then Nadia pretty much sat on his lap for the rest of the meeting and tired to comb his hair with some sort of play-dough toy. It was pretty cute, although in retrospect I should have taken the opportunity to go out for a coffee, it is obvious Nadia does not need her mother when she gets hurt.  sigh.

Team BMT were very happy with her counts and levels and put us down to one clinic appointment a week, whoop whoop.  They are also looking into getting her central line out in the next few weeks.  Which is pretty awesome as her noodles cause me some stress.  It can be a major source of infection, but if you are squeamish about IVs sticking into your hand or a kids hand try a central line bleh.  This is what goes through your mind when your kid has a central line, ‘is it gonna get infected? did I clean it properly? what if I go to hep lock it and I can’t flush the fluid into it? what if the line cracks?’ but the worst thought is, ‘what if it gets yanked out’ that thought is enough to keep you up at night.  Especially when you see the size of the tube coming out. It has the diameter of a juice box straw.  bleh. Nadia has adapted to it just fine, she barely even acknowledges it now. Which is great and she has been so great for a 2 yr old to have a (in my distorted mind) giant tube coming out of her chest. if you ask her where her noodles are she just points to her chest or pulls up her shirt a little bit and points to the ends of it.  It’s cute actually.

The dressing changes are a bit of a challenge, Nadia is pretty good, but she does cry and kick so it can make the process very stressful. I know it doesn’t hurt her really, just taking off the sticky dressing is the worst part. so I don’t blame her for getting upset.  She’s a trooper, once we are done she is back up and running around like nothing happened.

We have been spending as much time at the playground as we can when it is quiet and dry.  Nadia is loving it.  Up and down the slide, and not the small slide she has to go on the big slide, climb up to it herself, and slide down it.  I am still a bit scared of her falling and causing internal bleeding to herself, I think it will be a while before it sinks in that her platelets are in the normal range. Still paranoid about her touching the playground equipment with all the ‘germs’. If I could spray it all down with purell before hand I would, but they don’t make bottles that big, believe me I looked into it ;).

As I mentioned Thor left to go back to Kelowna for work last week. Even before Friday’s appointment we felt comfortable enough that it was time for Thor to do his semi-annual roadtrip throughout the BC Interior.  My parents came in from Calgary to stay with me to help and hang out, I wasn’t feeling 100% confident to stay on my own yet in case Nadia spiked a fever and I needed to take her back to the hospital. So it was great that they were able to drop everything to come and help.

Thor came back to Vancouver last night. We are happy to have daddy back.

On Tuesday I was able to do Nadia’s dressing change all by myself.  This is huge as it use to take 3 of us to do it in the hospital. One to hold down her arms, one to hold down her legs and the other to actually do the dressing change.  Nadia really has been a trooper through all of these types of things. Just before we were discharged she was pointing to the next step the nurses had to do, it was cute and disturbing at the same time. She screamed as I took the giant sticky band aid off her chest (as you would) but didn’t kick or try and touch the area. After I was done, she was up and running around again.

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Mmmm, toast with Nanny and Papa Raj!

Oh wow that TV is loud!

(ala blown away guy 😉

Climbing

Swinging like a big girl (she fell flat on her face right after, sigh). =Note from Thor: that shadow look right to you?=

Daddy daycare, sigh

Cookie Monstering Kale

AaaaChoooo

It will be one week tomorrow since, we were discharged, and Nadia has been doing really well. Her numbers are continuing to trend up, and we are so happy and grateful for this. Thor has updated the graphs for all to see, compare and get your nerd on.  As I don’t have a ‘math’ brain, I didn’t really appreciate the graphs, ok let’s be honest, didn’t really understand them, haven’t had much use for them since university economics classes and even then I would look at them like they were some ink blot waiting for a picture to jump out at me.  However, after this whole experience, I would HIGHLY recommend anyone who is in our position or similar to graph your counts.  Every day, we would get Nadia’s numbers, and every day when they wouldn’t move OR move down by .01 (which is nothing BTW) i would be devastated and stressed, then Thor would pipe up and say, ‘at least they didn’t go down’ even though I thought going down 0.01 was huge, it isn’t especially when you graph the counts. It wasn’t until one of Nadia’s doctors (who is a HUGE fan of Thor’s graphs, and they nerd out over them all the time, and now it has gone to Thor’s head) was saying to me “if you were to graphs Nadia’s counts, there is an upward trend…..” it was when the doctor said ‘upward trend’ that I realized I was focusing on the daily numbers too much and not looking at the bigger picture, which of course was an ‘upward trend’ and all I saw were numbers that went up and down a bit. Which when you are in the midst of something like this where numbers are important, it soooooo important to look at the bigger picture.  So if any of you who are reading this blog, and going through a BMT, and have become obsessed with the daily numbers (which you will), and need to look at it as a big picture, try graphing it.  Thor used excel and then did something fancy, as and engineer would, although I believe Thor is ‘over fitting the data’ (Thanks @IvesLevesque!) 😉 So thank-you husband dearest for nerding out over our daughter and thank-you doctor for those magical words ‘upward trend’.

We had our second clinic appointment today, it went well. Nadia’s platelets were 175!!!  When I think back to where they were at in September, 6, and pretty much that number until her BMT, I wonder how I didn’t have a nervous breakdown from worrying about her not falling and dying from internal bleeding! Now when she bonks her head or trips, we say ‘thank-god she has platelets’!

Nadia’s has been taken off one of her anti-rejection today, wooohooo! she has also started SEPTRA, which is a antibiotic, this is common antibiotic to give after a BMT, and they usually start it once your Neutriphil level hits 0.5, and as you know when we were discharged she was at 0.34. She has to take this twice a week.  We are also having to get more fluids into Nadia. The other anti-rejection drug Nadia is on, called CSA or cyclosporine, needs a lot of fluid to help flush it through her system, at least 1 ltr a day for Nadia.  So we have been trying to push a lot into her.  See in the hospital, she was on IVy and was being pumped full of fluids 24/7, but now that we dumped IVy (good riddance, insert a swear word that starts with B and rhymes with itch, here), it is up to Nadia to drink the difference. So we have been pushing water, juice, smoothies, and milk like some sort of meth dealer.  To the point where nadia gets annoyed and screams at us, then covers her mouth and shakes her head no!  haha!  she is so funny and such a 2.5 yr old.

We are enjoying home time/family time.  We haven’t had time like this as a family together since Nadia came outta the NICU, so we are enjoying it.  Thor heads back to Kelowna next week and my parents are coming up to stay with me while he is bringing home the bacon.  Then Thor will be coming back and forth between Vancouver and Kelowna until we get to go back to Kelowna.

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Just Nadia, looking adorable.

Testing out the new platelets. Look mommy, no hands!

A video of Nadia trial running her platelets.

Nadia showing off her lung function.