Nadia's Quest

Back in August Nadia gave a bone marrow biopsy, where they stuck a needle into her pelvis bone. They did this to determine if her bone marrow is capable of producing platelets properly, which would mean something in her body was destroying them. From this we learned that Nadia has very few megakaryocytes, so the problem lies in the bone marrow. But there is part of the bone marrow biopsy experience I would like to share.

When Nadia gave her bone marrow sample, Tara was holding her. They gave her some drugs through her IV. One of the drugs I think has the street name of ‘special K’. She was in Tara’s arms, scared, and after they administered the drugs she made her ‘o’ face. The one she is making at the time when she saw something that interested her, and she vocalized ‘oooo’. We think this was the moment she got high. Through the emotional stress and tears, we both found this amusing. A few seconds later she got heavy in Tara’s arms, but tried to hold her head up. It reminded me of the time Einstein was at the vet and he was drugged. Slowly drifting down like a tranquillized lion in the Serengeti.

I end this post with a Thank You to the staff at Children’s Hospital. They are great at what they do and sometimes they go above and beyond. Here is how we found Nadia’s bed when we came back from one of our outings. Cozy, cuddly, Scooby and Jelly.

Below is the contents of an email we sent to friends and family on August 26. This is before Nadia had a diagnosis, and before we were told that they would prefer, if possible, to keep Nadia from having a blood or platelet transfusion before the bone marrow transplant, because additional blood products could harm the outcome of the transplant.

Nadia update from Aug 26: She is doing well, and does not seem sick at all. But the bloodwork and bone marrow samples (and easy bruising) say otherwise. There are still a lot of unknowns, but so far it looks like Nadia is having some sort of bone marrow failure syndrome. Specifically, her platelet count is low and it is only a matter of time before the white and red blood cells follow. They have not narrowed it down precisely yet, but almost everything the do have on the list has the same treatment – bone marrow stem cell transplant. If they can better determine the cause they can better design the chemo that precedes the transplant.

They might send us home soon.

Because Nadia’s platelets are so low, there is a risk she could bleed into her brain spontaneously (unlikely but more likely than you or I), or after a severe enough trauma (likely – “no jumping on trampolines”). The more blood samples they take, the lower her platelet count has become. This is probably caused by her low platelet production ability (hence the low original count) and because they keep taking more blood from her (vampires!). They think they will soon have all the blood they need, so they will then give her a platelet transfusion and send us back to Kelowna while they finalize blood testing (samples are being sent all over North America to special labs, and this takes time) and look for a bone marrow stem cell match from an unrelated donor. I am guessing Nadia will need regular (weekly??) platelet transfusions until then. The reason they have not given her a transfusion yet is because they want to take as many blood samples as they can of her own blood in order to do the testing.

When it comes to bone marrow stem cells, Nadia is pretty much exactly 1/2 me and 1/2 Tara. So neither of us will be a match. The best option is a sibling, which she does not have. The other option is to take certain markers from her bone marrow sample and compare it to various international registries. We have had many people offer to be tested to see if they can help Nadia. Thank you all! But unfortunately it is very unlikely someone we know will be a match. BUT, I encourage everyone who can to enrol in the One Match stem cell and marrow network. They are keen on males between 17 and 35. It is a real challenge to get people into the registry because by the time most people experience an event that someone they care about (or themselves) needs a bone marrow transplant, they are too old to give. Even if you don’t qualify, please encourage those that do. You or they could end up helping a little girl just like Nadia. Entering the registry is easy. Fill out a form and if you qualify they will send you a kit so that you can take a cheek swab. That’s it. The One Match program is administered through Canadian Blood Services and they are included in the international network. You will only actually give bone marrow if and when they find a match (which, unfortunately, is unlikely). Here is the link.

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Of course, another way to help Nadia and people like her who need blood transfusions is to donate blood. http://www.blood.ca/

So it looks like we might be heading back to Kelowna soon. It also looks likely Nadia will need to be back in Vancouver for chemo, transplant, and recovery. It could take months. There is still a lot we don’t know.

On our second day pass we went to the Vancouver Aquarium with our good friends Cam and Suz. We did not realize that it was the weekend of the Stanley Park 125 birthday. Traffic in and out of the park was crazy. But it was not so bad with good company!

The aquarium was great, Tara and I have been there many times, but this was the first time for Nadia. She did not seem to notice all the fish. All she did was run around and up and down stairs. We could have stayed in a hospital stairwell and I think Nadia would have had the same amount of fun. 😉

We were blessed with a beautiful, sunny day. It was nice to be out and about.

On our first day pass we went to the Beaty Biodiversity Museum on UBC campus. It was awesome! I want to go back. We had to cut it short because Nadia had her fill. Daddy was being boring and reading all the signs.

There was so much to see and the blue whale skeleton overhead was soooo cool. And soooo big.

“How big is Nadia?” ….. “Soooo big”

Today Nadia turned 2. We had a small mommy-daddy-Nadia birthday party and ate cupcakes. On the weekend Tara’s mom was in town and we had a pre-birthday cake. We are having a post-birthday cake next weekend when my parents are in town. Today when Tara asked Nadia who’s birthday it was, she said ‘cake’. It is fair to say Nadia likes cake, here is some proof:

http://youtu.be/lO02K9OQpVA

Post birthday celebrations was cleanup time. When we try and use a broom Nadia grabs it away from us. It is great that she is helpful, but she is a bit reckless with the top of the broom. So yesterday I bought another broom and cut it down for her. I made a wooden plug for the end and wrapped it in good old hockey tape. Somehow this process gave the broom magical qualities, which Nadia was quick to figure out.

http://youtu.be/tjpnv6hpJ7U

Happy Birthday Nadia!! Cheers to many more returns.

Just Like Home Dog Care is amazing and Thank You, Thank You, Thank You to Kim and Neezar for being amazing and so accommodating, taking Einstein in on such short notice. We left Einstein with them once or twice when we last lived in Vancouver, and they remembered him. They were super-kind and understanding, letting us leave Einstein with them without knowing when we would pick him up. It really took a lot of added stress off our shoulders and we knew Einstein was in good hands and would be having a lot of fun with all the other dogs and the 1 or 2 walks a day. So Thank You Kim. Thank You Neezar. And Thank You Just Like Home Dog Care.

Thursday and Friday consisted of being patient and taking blood samples (vampires!), and a bone marrow biopsy from Nadia’s hip. Unfortunately, there was one more blood sample to take and they can only take these types of samples on Mondays and Tuesdays, so we had to stay in Vancouver until the last sample was taken. Children’s gave us ‘day passes’, but wanted Nadia to spend the nights in the hospital in case her blood levels decreased, because at that time they still did not know the cause of her low platelets.

Nadia took it all in stride though. She had an IV in her arm and her arm was taped to a board, making her hand unusable and kind of like a club. But she did not seem to mind.

Children’s has some amazing play rooms and Nadia wanted to spend all her time there. At first her IV was attached to a pole, and it was comical trying to keep up with her in the play room. She would run around with one of us trailing behind with the IV pole and Nadia would, seemingly intentionally, dropping obstacles in the way, at which time the other parent tried to clear the path. It was much like a chase scene from a cartoon (maybe Scooby Doo??). It was hard to keep up, but if she got too far ahead or snagged a line she could rip out the IV.

It was amazing how well she coped. I thought she would get grumpy by having one hand taped to a board and would try to get it out. But she seemed content playing using her free hand, and using her bound hand to hold things by cradling them in her arm, to her chest.

http://youtu.be/IUZw3TUw2KA

Besides getting poked with needles and freaking out whenever someone came in the room who had a stethoscope around their neck, I think Nadia had some fun. She has never seen so many toys in one place at one time. And she got to play with some other kids.

At all times Nadia insisted on wearing her shoes, and her hat. She is a very colour coordinated little girl. How many shades of pink do you have on?

I don’t remember much about the drive to Children’s. I have a feeling we were pretty quiet and Nadia almost definitely watched Scooby Doo the whole way. We left Kelowna after 2pm sometime and ended up in Vancouver well after 6pm.

During the drive we remembered the dog boarders we used when we last lived in Vancouver, Just Like Home Dog Care. So we had a solution for the dog, we just had to contact them, hope they had an opening, and get Einstein there.

Just to add some perspective, Nadia is a great sleeper and is usually in bed just after 6pm. She typically stays down for the night. We arrived at Children’s at about the time Nadia goes to sleep. We went directly to Emerg, as we were instructed, and all of Nadia’s paperwork was waiting for us.

Then the doctors came through. One or 2 at a time, spaced 30 to 40 mins apart. I don’t remember any of their names or what types of doctors they were. Bloods were drawn. We definitely saw hemotologists (blood doctors). This was the start of the vampire hunger the hospital would have for the next week. But I guess it is not too surprising that blood doctors want blood to look at.

It was very hard. So many unknowns. Nadia was overtired and screaming whenever someone came in the room who had a stethoscope around their neck. And during this whole time our dog was stuck in the car in the basement parkade.

Fortunately it was not hot outside and it was even cooler in the basement, but Einstein was still on our minds. We also worried that some over-concerned citizen would call the SPCA or something. Or maybe the police. Or maybe they would just be ‘heroes’ and break him out of there. I admit it was a bit cruel to leave him cooped up in a car as long as we did, but it was certainly not inhumane. He just would have been bored. We know our dog well enough to know his limits. But we were worried about the added hassle and stress a well meaning, but frankly ignorant, person could cause.

At about 10pm we decided poor Einstein had enough and I took him for a walk. It was a hard thing to do because we never knew when the next doctor would come in with more information or to ask more questions. I did not want to leave Tara alone.

It was not too long after I got back from walking Einstein that the doctors made the decision to admit Nadia to the hemotology/oncology ward. The were confident from the testing so far that it was unlikely Nadia had leukemia, but the still had no idea what was causing her platelets to be so low. I helped settle Nadia and Tara in to the room, and left to spend the night in the condo with Einstein. Nadia finally go some overdue sleep and Tara and I tried to get the best sleep we could to face the next day. To face the unknowns. To hope for the best.

My only parenting advice (completely unsolicited) to my brother was, “it gets easier every day.”

It is such an unpreparable experience having your first kid. The sleepless nights, extra chores, loss of personal time, sleep deprived decision making, ‘kids are gross’, lack of sleep (did I mention that already?) – it is all quite an adjustment. But it gets easier. It gets easier every day. It quickly becomes rewarding.

That is, unless it gets harder.

Tara and I are not (well, were not) paranoid parents. We are not overly-concerned parents. We were almost laid back parents. Getting hurt is part of growing up, and it is best for kids to learn their limits instead of growing up in a bubble and ending up ill-equipped for the real, hard edged and sharp cornered world. So when Nadia started walking, bruises were expected.

She seemed to bruise easily. But some people bruise easy. Her nanny bruises easy. This is normal. Thankfully, Tara is a little more of a concerned parent than I.

Nadia ended up with a huge hematoma on her side in late July. Daycare did not know where it came from and neither did we. I poked it with my finger and it did not seem to bother Nadia – it was not painful. I have had hematomas like that and they hurt. If it was up to me I would have left it at that, my reasoning being if it did not hurt her it can’t be bad. But Tara recognized this as strange, and also noticed some purpura, so she made a doctor’s appointment. Tara was scared Nadia had ITP and I thought Tara was crazy. The doctors would set her straight.

It took a couple of weeks to get into a doctor appointment. By this time the majority of Nadia’s hematoma had gone away, but there were still some purpura. The GP decided to make a referral to our pediatrician, and ordered a blood sample. The appointment was almost a week away.

On Wed Aug 21 Nadia gave a blood sample in the early morning and we met with our pediatrician at noon. Now the pace changes. It is not ITP. Our pediatrician was not happy with the bloodwork. He circled a bunch of numbers. They are too low. Specifically, Nadia’s platelets were low and this explains her easy bruising. We needed to determine the cause of the low platelets. It was possible that Nadia had a virus or something that was attacking the platelets after they were made, in which case she will recover with time. More sinister, she might not be making platelets properly. (he did not say it, but could it be leukemia??) Either way, we needed to go to Children’s hospital for further tests. Given Nadia’s apparent well-being, there was no need to air lift her, but we needed to go TODAY. (it was already noon and Children’s is more than a 4 hr drive away) Our pediatrician told us to go home, pack our things, and drive to Children’s. I asked how long to pack for, and our pediatrician said to pack for a week, giving us a clear between-the-lines look telling us that he did not know. But if we planned for a week, then we would be well prepared to stay longer.

That left the dog. What to do with a dog when you have to leave town immediately, for an undetermined amount of time, and will be mostly at the hospital.

This was the beginning.

On Tuesday, Nadia had a cold. Throughout the day her temperature was high, and went up a bit. Just high enough to be a fever. Our instructions are to take Nadia to the hospital if she has a fever, no matter what.

So we did.

Nadia has her own binder at the hospital with specific instructions on what to do if she requires a transfusion. If she needs a transfusion they must use blood that has been prepared in a special way in order to minimize her future risk when she has the bone marrow transplant. The fewer the blood products the better.

So it is kind of a stressful thing to consider that there is a possibility this fever from a simple cold could result in a blood transfusion that could negatively affect her bone marrow transplant outcome. But we can’t take a chance.

So we go.

It took a couple of hours for the doctors to see her, take blood, and get the results. Fortunately her blood counts were OK. This will just be a ‘normal’ cold.

Thankfully.

She appears to be much better today.

As you can imagine, we were caught quite off-guard with our daughter’s diagnosis, and life has been very hectic since. Having been a blood donor for the past year, I was very surprised to learn about Canadian Blood Services’ OneMatch Stem Cell and Marrow Network. I had never heard of this program and would have volunteered long before learning my daughter needs a bone marrow stem cell transplant. Our friends were equally surprised.

I think more people would volunteer to be bone marrow stem cell donors if they only knew this program existed. We are hoping our family’s experience will help raise awareness. With the help of some great friends, we have started nadiasquest.ca and a facebook page. The process is simple, you fill out an online form on the OneMatch website, they send you a swab kit, and you mail it back. Like my brother said, “The hardest part was remembering to do the swabs 30 mins after eating.”

I will soon post some correspondence we sent to family when Nadia started her quest. Back when life was normal and our daughter bruised easily, but we did not know any better. Back before we knew our daughter was in bone marrow failure. Back before I thought telomeres were only of concern to vain baby boomers. Back before we were able to spell, and never even heard of, dyskeratosis congenita. It seems so long ago.

But before I do, lets start on a lighter note. Here is a video of our bruised beefeater that delighted us all.

http://youtu.be/3H0wge4O67Y