Dear Oakley

Dear Oakley,

Your room, waiting in anticipation for your new bone marrow

 Today is a very important day! It's your day zero, your start over day. Today you are getting a whole new immune system. Your dad and I have been so excited for you that we didn't really sleep (we each took about an hour nap), because we didn't want to miss the bone marrow's arrival. You've been in the PICU for 5 days  now, and we've been sad to see you hurting from your chemo conditioning. One of your chemotherapy drugs made your lungs take on a little extra fluid, and made it kind of hard for you to breathe. You've been on oxygen off and on the whole time you've been here (and it's put a damper on your Halloween week outfits), on a regular nasal cannula, a high-flow cannula, and CPAP.

Look how dark red it is

Your bone marrow transplant infusion started at 5:27 AM, and when the BMT team brought it in, they sang Happy Transplant Day to YOU (it's a lot like Happy Birthday, but a little more special-everyone has a Birthday, but not everybody gets a whole new immune system, and a second shot at life given to you by a stranger). We don't know very much about the person who donated part of his immune system to you. We will find out more about him in a year; but for now, we know he is 23 years old, is a boy, has O+ blood type (and now you'll change to that from A+), and he lives in Europe. Your transplant was supposed to get here yesterday evening, but there was a hurricane on the East Coast of the United States called Hurricane Sandy that delayed the marrow in getting to you as soon as possible. Once it arrived in Seattle, it had to go to a lab, where they took the bone marrow and put it in a machine called a centifuge that spun it around and around until some of the clear liquid separated from the red blood cells, so they could drain them off to make it more dense.

Here it is!

As of a few hours ago, you weighed 6.35 kg (which is 14 lbs exactly), but you've been growing so well, they used an old weight when they got the transfusion. You are getting 126 mL of blood cells, and you're still so tiny that they have to give it to you really slow; it's going to take over 4 hours!

before your transplant

We love you so much, buddy. We are so happy that you're young enough that you won't remember any of this experience; because even though there has been a lot of great stuff (awesome people who've come to love you, who've saved your life, who've helped keep Mommy and Daddy sane), there has been a lot of tough stuff you've gone through too. It's not the end of either for us, dude, but we're going to make sure we fight with you the whole way through. You haven't lost your hair from the chemo yet, you're supposed to get some pretty nasty rashes soon, and there's a pretty high likelihood that you'll get something called GVHD (that stands for graft versus host disease) that will make you sick. The whole way through though, people comment on how cute you are, because despite all you've been through, you've given smiles and coos to nurses and doctors and have made their days.
You've got a lot of people rooting for your success buddy. They've been praying for you to get through the whole thing, because several times, you've given us some scares that you wouldn't make it. There are people all over who are supporting you with their thoughts and prayers, and that has given us a lot of hope.
Well, we're going to update your grandparents and uncles now, we love you and are so proud of how strong you've been.

I tried to dress you for the occasion; you had other plans

Your 2nd outfit

See the blood cells going into the red lumen? That's your
new bone marrow!

-Mom and Dad

Day of Rest

Yesterday was Oakley's last day of chemotherapy! He has done far better than Trevor and I expected and prepared ourselves for. He had very little pain, and wasn't too nauseated either. He only vomited a few times, which is less than on a regular basis! We are still headed into the waiting period where his body has to accept the transplant, and the transplant has to accept his body as it's host, so there is still a lot to come. He hasn't lost his hair yet, he hasn't gotten the horrid skin rashes and diaper rash that is apparently inevitable.



try to ignore the lipskin and focus on his teeth



This morning, he did have some mucusitis starting up; which means his system is going to be shedding it's lining. He sounds awful, and has so much phlegm that they ran cultures again, just to ensure this isn't an infection. I'm kind of sick of them doing those every couple of days...this time he doesn't even have a fever...why would you figure it would be an infection, and not the expected side effect of his conditioning?



CPAP; looks like a rhino huh?



As Miranda pointed out, I must have forgotten to update the blog that Oakley has been teething. His second tooth broke the skin on his 6 month birthday, so he's had two teeth for almost ten days and I haven't loaded pictures yet! So sorry! He really is growing up right before our eyes, and I just wish he'd slow down! I'm not ready for him to grow out of his baby stage, but he's trying to sit up, he's rolling to his sides to get better views, and he understands some of what we say. (He kicks on command and is trying his hardest to give high fives now. He also kind of smacks his lips when we kiss him.)

He's pretty dextrous now too. He's pulled his NG tube (the tube that goes down his nostril to his tummy-he got it so we can give him his meds without fighting him) a few times, he actually puked it out once. His nasal cannula has been through the ringer, because he can get that thing off with his hands mittened! Oakley's an expert hair and beard puller too.

or maybe a snork?

Tomorrow is transplant day, but since the marrow is coming from Europe it'll be here pretty late in the day, and then it has to go through testing in the bloodbank before we actually get it here in the hospital. It will probably be given in the wee hours of Tuesday morning. Everyone here says that bone marrow transplant days are second birthdays, so Oakley can't hear of this, it's too close to Christmas time for us to give him this second birthday!

We are still in the PICU, and noone has really talked about any desire to get him out of here and back on to the SCCA floor, so I don't know if Oakley will get to don all of his Halloween costumes and outfits. He's allowed to wear two for now, because they button all the way down the front.

The other bummer about the PICU is that only one parent is allowed to spend the night, so Trevor and I have been drawing straws as to who gets to sleep on the sleep number bed across the street, and who stays here and is here for rounds in the morning and guards Oakley from being intubated for practice. 

Well, we'll keep you updated as the process drags on, thanks for keeping us in your prayers!

Love,

Sam, Trevor, and Oakley

P.S - Trevor here, we are super excited to have our lil dude go through this next part and if his tenacity and ability to be a rotten lil fighter is any testimony of how he'll handle the next few rounds of what is coming, I think he'll do just fine. Things haven't gone as we had thought the last few days or even weeks, but then again, none of the last six and a half months has been to the T. We can't wait to take him outside, put him in his stroller, and promptly drop him in a mudpuddle. He deserves it. Believe me. We miss Idaho and cannot wait until we can be back with you all!

Also, please remember to vote. 

Love, Trevor. 

and Sam. 

Oakley's asleep right now. 

Just now, enjoying his day of rest

Chemo

Hello all! Oakley started chemo on the 23rd. His first day he had (and I apologize in advance for misspellings) fluaderabine and treasulfan. The regimen that he is undergoing is a clinical trial, as the treasulfan isn't fully approved by the FDA in the US. He reacted quite well, didn't get sick too bad, and was in a pretty awesome mood. The only thing noticable was that they were having to chase his blood sugars around a bit. The second day was the same routine-fluaderabine and treasulfan. He wasn't as happy, but still not nearly as sick as we had braced ourselves for. His sugars were higher, and they were chasing them still, and unable to get ahead of them. He got a fever in the afternoon, but still seemed like he was doing well. The fluderabine can cause feers, and fluid shifting, and the treasulfan causes nasty diaper rash, but he really only showed fever thus far. That night, as Trevor and I slept, Oakley's sugars continued to outrun the team on the SCCA (Seattle Cancer Care. Alliance-bone marrow transplant ward of Seattle Children's) floor, so we were woken up to a nurse telling us to pack up, we were moving to the Intensive Care Unit. Oakley had started breathing quicker and still had a fever, and was so high on his insulin pump that they couldn't do his dose within their guardrail. Basically, they sent us to the ICU over .05 cc's of insulin per hour. They could have just updated his weight in the pump, but instead, they sent us to ICU. They used the excuse that they might have to intubate him at some point, and they didn't want to have to do it in a hurry. (I'm still bitter about all of this. I feel like everything was in their scope of support, ye they sent us up to ICU because they're lazy and not savvy enough to deal with diabetes)
Once we got to PICU, they ra all of the usual tests to check for infection. They did blood and sputum and stool tests, all of which came back negative. They said they wanted to do a CT scan, and they told me they wanted to intubate him for it. This was a stupid request in my opinion. They wanted to be able to inflate his lungs in the machine at will-but the risks associated with putting a tube down a kid's throat, opening a passage for everything to get into his system-especially one who has a freshly obliterated immune system-is too big of a risk. The risk to benefit ratio on this is nearly 100 to 0, becaue they hadalready started him on broad-spectrum antibiotics, anti-virals, and anti-fungals...so even if they found something, they wouldn't change what they were already doing. The pulmonologists came in and said they wanted to do a bronchoscopy, so put a tube down into his bronchial tubes and lungs, with a fiber optic camera, and flush it with saline and then test the fluid. They said he's need to be intubated for that as well. Trevor and I talked it over, and pretty much told the doctors that we know our kid, he doesn't have an infection, this was his chemo, and that intubation wasn't a viable option at this point. The bronchoscopy would be to search for fungus, but his symptoms came on more quickly than typical fungi, and we weren't certain he needed it to look for something that was already being treated prophylactically. The pulmonologist was a greasy sleaze bag, who tried to treat us like we were dumb, and really pulled some used car salesman tactics on us. "I know you guys just want to be the best advocates for your child, and I repect that, but I think you don't realize that we just want to intubate him temporarily. This won't be a permanent thing."
Really? You mean you just want to shove a ladder for diseases to get down from his mouth to his lungs for a little while-just while has absolutely zero immune system? Well in that case, no.
"Well, I'm not a bone marrow transplant expert, but the BMT team really wants this test to be done so we can find out the reason your little guy is here."
The reason he's here is sugars,  and then a fever and heavy breathing from his chemo. They can come talk to us and tell us to our faces if this is required. You can CT him without intubating-you did last week.
"We would like to do a prone view-what that means mom and dad, is that we want him to lay on his tummy, so we can get a full view of his lungs. When he's on his stomach, we can see a different view than when he's on his back."
Really? You mean when you look at someone from one direction, and then flip that persn over, you can see a different view? No, I don't believe it. Is there something in particular that leads you to believe that Oakley is incapable of being laid both on his back, and then inversely, on his stomach without a tube down his throat?
"Well, we just can't make him hold his breath, so it probably won't work. I'm really just trying to help you be advocates.  I know things like this can be scary, especially when we don't have answers for you yet."
You mean why he has a fever? Or why his breathing is more rapid? Couldn't possibly be that the kid's red bloodcell counts are low and he's gone through two days of chemo, and is halfway through the third, could it?
By the way, day three was fluderabine and then ATG from a rabbit. ATG is terrible stuff, but he seems to be doing alright so far.
"Let me see if we can put an LPA in, that's a disc that blocks the airway from the digestive tract, and we can do the CT like that?"
Why does he have to have anyting? He does just fine in them without mechanical breathing.
(This went on, to the point where Trevor and I just spoke frankly, telling the doctor that nothing is wrong with Oakley, other than the fact that he has IPEX and is on chemo. Also saying that this was a ridiculous and unneccessary test, that was being proposed because doctors are innately unwilling to admit they don't know something.
We did the CT with no breath support other than his nasal cannula. It came out great, and clear. The BMT docs told me they never approved and definately didn't request that Oakley be intubated for a procedure. They told us they didn't even think the bronchoscopy was necessary, but the pulomonlogist suggested it, and they reponded that if, for some reason, Oakley's breathing got to the point where intubation would be necessary, then they could go ahead and do the scope.
Anyway, Oakley's doing well. His sugars have been more stable, but are bouncing around a lot and are expected to through this process. He's still on some respiatory help, they put him on CPAP today, but is doing very well.
Tomorrow is the last day of chemo-Rabbit ATG and fluderabine I believe, and I'll be so happy to be done! We get Sunday as a day of rest, then bone marrow day is Monday, the 23rd!
He's expected to lose his hair next week, as the drugs hang in his system for a while.
It's difficult to see the light at the end of the tunnel if you do not have the faith to enter the tunnel's mouth. We are in the tunnel, and the light from our surroundings is getting blocked by the cavernous walls and ceilings of isolation, chemotherapy regimens, and medical facilities, but we are in the tunnel, with faith that we will see the end soon.
Oh, side note, they won't let me nurse Oakley until after engraftment and he has leukocytes...so it could be months before he gets any breastmilk again.

Half Birthdays

taking a nap in the sunshine

Friday, my baby turned half a year old. It's not fair. I feel like he shouldn't be allowed to age so quickly, especially since I haven't gotten to even take him home yet! I feel like most of the stuff we got for Oakley won't ever be used, and that makes me so sad; but not for Oakley, for me.

I am bummed I don't have newborn pictures of him. I'm sad that he won't fit into his bassinet and even if he does, he'd tear it apart.  I'm so happy we went with a convertible car seat, because he would be too long for an infant seat soon, and we still don't know when he'll ever get to go on a car ride (ambulances don't really count).

Thursday, I cried for the first time in a while. (That's not true, I cry all the time; but this was in front of someone, so it was different) The doctors put Oakley back on his zenpep (his pancreatic enzyme) because the tests came back and his exocrine function is not as good as they thought it was a few weeks ago. The old argument of apple sauce came up. Now I am aware that this is a stupid fight to have. Obviously, I should let them give him his zenpep in applesauce, however, food means so much to me. It is the one thing I think should bond a mother and child, and I feel like I was gyped in that area when he was born. I really don't want food to be a part of any medical procedure. I really want that to be something special, and even though we've let him taste our food every now and again, I don't want applesauce to be in the equation.

The doctor told me she didn't want him swallowing the capsule whole, because she was afraid he might choke, and doesn't want anything to postpone his transplant. She didn't want to give it with his meds, because she doesn't want him to spit up his meds with the beads, and she didn't really give a reason I can remember as to why she doesn't want to just chase the beads with water.
I reiterated my feelings about giving him applesauce as a part of a medical procedure, and I got pretty choked up. I told her that this was my one shot. I only get one baby because I'm a carrier for a deadly disease, and I wouldn't want to pass that on to another person. I said that I felt like I had been robbed of so many things already, and I didn't want this to be taken away from me too. It caught the doctor off guard, and she dropped the applesauce request and conceded to chasing it with cherry syrup (A real-live cherry chaser Miranda!). She didn't want to give it with his regular meds, because she didn't want him to spit his meds out, and she didn't think water was going to help get the beads down without causing a choking hazard, so here we are.

I should treasure it though, because she told me that once chemo starts, they don't want Oakley to receive breastmilk at all. I've had CMV, and so has Oakley, but they fear that it will introduce it to his nonexistant immune system if we nurse. After today, it could be months before we get to nurse again.

We had our review on Thursday afternoon, and Oakley's donor is a 23 year old male from Europe. We are doing a 5 day course, and are in a medical study. It's a medium-high regimen, so there is some worry that Oakley's tiny body won't be able to tolerate it. The doctor actually expressed concern that he might die; which surprised Trevor and I. We have had so much hope and have been looking further into the future (since all we really focus on is that he will be cured with the bone marrow transplant) that we kind of forgot about the risks of getting there. I haven't been this scared in months.

Oakley's tree is losing it's leaves in
preparation for Oakley to lose his hair

We are going to enjoy today as a family; looking out the window at Oakley's balding tree, as we prepare for Oakley to lose his hair. Enjoying the changing season, as we brace ourselves for the changes we are about to encounter.

We'll try to keep this up to date, but try to understand if it gets pushed to the side from time to time.

In the meantime, thank you for keeping us in your thoughts and prayers. We appreciate all the love, support, and generosity we have received.

Love,

Trevor, Samanatha, and Oakley

Taking the Good with the Bad

Not much to do here

Hey friends, family, and people who've heard our story! Just wanted to give you an awesome update, and unfortunately, it's coupled with some not so awesome information. Awesomeness first: We have a transplant date (FINALLY)! Oakley has reached his 6 KG goal (he got up to 13 lbs, 4 oz)! The doctor finally gave him a date and he is set to start chemotherapy or conditioning, on the 23rd of this month, with day zero, or transplant date of October 29th. They will be doing a 5 day conditioning regiment, 1 day of rest, and then the transplant. We are so excited to actually have the ball start rolling, so we can count the days until we can take him out of the hospital. I cannot wait until he can feel grass on his little feet, see a bird-even if it is a crow or a pigeon, and pet a dog! There aren't service dogs in the bone marrow transplant ward, and there won't be any in the apartment that we'll be staying in once we're outpatient, so I don't know if we'll just wait until we go home to Boise and he will see his dog for the first dog experience, or if we'll have to sneak him a dog to pet beforehand. I also can't wait until he's outpatient so we can live as a family, in the apartment for the first time. Trevor and I stay at the hospital with Oakley, so it's almost like we're living together, but it's going to be so awesome to not have people coming in every 1-2 hours to do things that Trevor and I could honestly do ourselves.

taking a quick breather at Lake Washington

I would venture to say that we are much better at blood sugar checks than the nursing staff here, and we are able to interpret the results and the necessary steps to ensure he stays in a good range or gets to a good range. Trevor has hospitalitis. He is starting to resent and loathe the nursing staff here. I can understand it, because several of them seem to be inept, but they are trying. If you've ever had kids, you may remember how everybody wants to give you parenting advice, and that for some reason, they think their idea is unique and amazing, even if it's something you've already been doing. Our experience with the nursing staff here is just like that, but several times a day. It gives us something to talk about though. For instance, Oakley has been on med pumps since day 1. Med pumps alarm when: there's an obstruction (like a kink in the hose, or if you roll the wheel over the tubing, or if it's clamped) when it's supposed to be running, the battery is low, there's no more medicine left in the syringe or in the bag (meaning the infusion is complete), or if you set the med to go for 150 mL and you need to reset it to keep going. Trevor and I are very comfortable with these alarms, and are capable of silencing the pumps because sometimes the nurses are busy when they go off. We can even restart the pump if it gets an obstruction, and we fix it. I could even shut the pump off when the med is done, but I shouldn't, because I'm not a nurse. Despite all of this, our nurse yesterday told us that we shouldn't worry if the pump starts to go off, because it's just one of his medicines beeping. We were surely wearing our irritation on our faces, but just said, "Yeah, the tacrolimus." The tacrolimus goes off everyday, and has for about 3 or 4 months now. We're pretty familiar with what time he receives it, and that the pump alarms when it's done running. For the rest of the day, Trevor and I joked about this (and other remarks the nurse had made that were very common knowledge for us) for the rest of the day. Hey, did you know you're not supposed to leave your baby face down in a tub of water? Did you know you're not supposed to put plastic bags over their faces? No, of course we didn't know that...bummer, I had a whole stash of Walmart bags sitting at home for Oakley to play with. (Obviously we're sarcastic about this, don't call CPS)

Sorry for meandering, because that's actually not the bummer news. There are two real bummers, the first one is that the TGI Fridays bartender competition that was going to benefit us by giving us the proceeds is now called off. Apparently, one of the two ladies running the charity night quit her job, and the other one had a family emergency is on leave. These two were so involved, that the rest of the folks at TGI Fridays didn't even know how to get in touch with us to let us know. My parents went to lunch there Sunday, to support them since they were going to be supporting us, and they found out about it. I'm planning on getting back in touch with the manager who is still there this week, so I can find out if they're just postponing it, or if it's completely off.

The second is, I finally lost my job. I have been on a leave of absence for my own medical issues (which still persist...I have 5 doctor appointments this week alone), and because I have been out of the workplace for so long, they had to let me go. I am super bummed, not only because our family will sorely miss the insurance that T-Mobile provided, but because all the benefits were awesome. I liked my job, had been there for over 5 years, and will miss it. I will also miss the awesomely cheap cell phone plan and getting my new phone every year for free! I am re-hirable, so I will go back to work there once things quiet down-assuming we've got things figured out for Oakley.

We're trying to figure things out, to see how to make life work for now, but in the meantime, I'm learning to figure out the shuttles so we don't have to pay for gas (it's over $4 per gallon here!), and to avoid allowing my car the opportunities for mechanical failures.

Keep us in your thoughts and prayers, as we enter the rigors of pre-conditioning, chemo, and the transplant process. I especially hope that you pray for our mental well-being, as it's taxing being isolated from the world, and we are under tremendous amounts of stress, and for Oakley to continue to gain weight, and keep up his appetite during the conditioning and transplant processes.

Sorry for the lack of blogging!



 View from the solarium



We've had a fairly boring time; Oakley's been doing well, and is finally gaining some weight. As of this morning, he weighed 5.74 kg (which is 12 lbs, 10.4 oz). Great news from yesterday, the little guy doesn't have to take his Zenpep anymore! Those are the pancreatic enzymes he has had to take for over 3 months, and he had to swallow them either in bead or capsule form. He had gotten pretty good at taking pills-especially since he's just a baby still-but I'm so happy that he no longer has to use that skill multiple times a day. The reason that he doesn't have to take it, is that his last stool sample had a normal fecal elastase reading, meaning the enzymes that his pancreas is supposed to emit for digestion were in there, so his exocrine system is working! This is so exciting, because the exocrine portion of the IPEX syndrome was kind of an unknown as far as if it would reverse when he finally gets his bone marrow transplant.

Update on the BMT: nothing. They're supposed to re-evaluate him, but we haven't heard any real news, other than his current doc is "guessing" they'll start conditioning (chemo) in two or three weeks.


the other view from the solarium



Today we had an interesting day...Oakley's TPN (his total parental nutrition) tube broke in the middle (in a joint where it goes into a filter) and was spilling all over...and we don't know for how long. I realized it when my jeans were soaked and smelled like Easter egg dye, and there was TPN everywhere. We called for the nurse, and in the meantime, checked his blood sugar with Trevor's glucometer. His blood sugar was 38, which is bad. He had been pretty cranky for a while, so we didn't know how long he'd been low, so we fed him while we waited for the medical staff to take action. By the time they came to even check his blood sugar (they came and turned all the pumps off, so he was not receiving anymore insulin), he had come up to 58, so he had made a pretty good jump on his sugar with his feed. They gave him some D10 (which is 10% dextrose) about half an hour after they knew his TPN had broken. It was weird, because after the doctor wrote for the D10 almost immediately, and was checking on Oakley and didn't realize it hadn't been run. The nurses had been arguing and calling pharmacy to find out how much time to run the sugar water over, instead of just giving it to him. He got spacey a few times, but never lost consciousness or had a seizure, which is the worry with low blood sugars. I felt so lucky to have Trevor there, so he knew what to do and we were ahead of the staff in caring for our kid.



where Trevor and I sleep, and we have
family cuddle time with all 3 of us



Endocrinology wants Oakley to start on a continuous glucose monitor once he's 6 months old. That means on the 19th, we'll put in an order for one and hopefully not have to stress out about highs and lows due to malfunctions sneaking up on us. I'm excited to get one, because the hospital won't actually monitor it, Trevor and I will be responsible for it, and then it's one step closer to actually being wholly responsible parents.



the TV and some of the drawers



I've been busy working on a slideshow for the TGI Friday bartending competition that's coming up, so haven't really had time to work on the blog, but Trevor's mom and her boyfriend, Bill, were in town over the weekend, so we got some time out of the hospital. My cousin, Crystal, even came and hung out with Oakley so that the 4 of us could spend some time out together. It was a fun-filled weekend of exploring North Seattle for Trevor and I.

 The best thing that happened this week though, was that we got our GIANT ROOM back! Yay! This means Trevor and I can sleep without spooning. It also means that Oakley has his view of his tree, and his whole solarium play room back! We were so excited that we have been sleeping like babies! We've also been able to watch the leaves as they slowly start to turn yellow. It also means we have ample space for Kinect, so we've moved our XBOX in and been playing darts, golf, and dancing!



Oakley, enjoying sunshine rays through his tree